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Williams Hematology
Malignant
Lymphoid
Diseases
Oliver W. Press, MD, PhD John P. Leonard, MD
Giuliani/Press Endowed Chair in Cancer Research Richard T. Silver Distinguished Professor of Hematology and
Fred Hutchinson Cancer Research Center Medical Oncology
Professor of Medicine and Bioengineering Joan and Sanford I. Weill Department of Medicine
University of Washington Weill Cornell Medical College
Seattle, Washington New York Presbyterian Hospital
New York, New York
Marshall A. Lichtman, MD
Professor Emeritus of Medicine
(Hematology-Oncology) and of Biochemistry
and Biophysics
Dean, Emeritus School of Medicine and Dentistry
University of Rochester Medical Center
Rochester, New York
New York Chicago San Francisco Athens London Madrid Mexico City
Milan New Delhi Singapore Sydney Toronto
ISBN: 978-1-26-011707-3
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William J. Williams, MD
1926 – 2016
Medical educator, investigator, physician, mentor, academic leader,
colleague, and the founding editor of Williams Hematology
3 4
5 6
1. Transmission electron micrograph (TEM) of a normal blood lymphocyte. 2. Scanning electron micrograph (SEM) of a normal blood lymphocyte.
3. TEM of Sézary cell in a patient with the erythrodermic type of cutaneous T-cell lymphoma. Note the cell’s characteristic profoundly misshaped
(cerebriform) nucleus. 4. TEM of a hairy cell. Arrow indicates a ribosome-lamella complex. This structure is not specific for hairy cell leukemia but is
found in a variable proportion of hairy cells in about 50 percent of cases examined by TEM. Frequent cytoplasmic membrane, “hairy,” projections.
5. TEM of plasmablast (undifferentiated myeloma cell). Arrow points to a Russell body. 6. A lymphoblast from the marrow of a patient with acute
lymphoblastic leukemia. Very high nuclear-to-cytoplasmic ratio. Prominent nucleolus. The nucleus is virtually all euchromatin (likely transcriptionally active).
(Reproduced with permission from Lichtman’s Atlas of Hematology, www.accessmedicine.com.)
CONTENTS
Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii 12. Marginal Zone B-Cell Lymphomas . . . . . . . . . . . . . . . . . . . . . . . . 175
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi Pier Luigi Zinzani and Alessandro Broccoli
13. Burkitt Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183
1. Classification of Malignant Lymphoid Disorders . . . . . . . . . . . . . . 1 Carla Casulo, Jonathan W. Friedberg, and Andrew G. Evans
Robert A. Baiocchi
14. Cutaneous T-Cell Lymphoma (Mycosis Fungoides and
2. Acute Lymphoblastic Leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 Sézary Syndrome) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 191
Richard A. Larson Larisa J. Geskin and Christina C. Patrone
3. Chronic Lymphocytic Leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . 37 15. Mature T-Cell and Natural Killer Cell Lymphomas . . . . . . . . . . 207
Farrukh T. Awan and John C. Byrd Neha Mehta-Shah, Alison Moskowitz, and Steven Horwitz
4. Hairy Cell Leukemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63 16. Plasma Cell Neoplasms: General Considerations . . . . . . . . . . . . 223
Michael R. Grever and Gerard Lozanski Guido Tricot, Siegfried Janz, Kalyan Nadiminti, Erik Wendlandt,
5. Large Granular Lymphocytic Leukemia . . . . . . . . . . . . . . . . . . . . . 73 and Fenghuang Zhan
Pierluigi Porcu and Aharon G. Freud 17. Essential Monoclonal Gammopathy . . . . . . . . . . . . . . . . . . . . . . . 237
6.
General Considerations of Lymphomas: Epidemiology, Marshall A. Lichtman
Etiology, Heterogeneity, and Primary Extranodal Disease . . . . . 81 18. Myeloma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 249
Oliver W. Press and Marshall A. Lichtman Elizabeth O’Donnell, Francesca Cottini, Noopur Raje,
and Kenneth Anderson
7. Pathology of Lymphomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99
Randy D. Gascoyne and Brian F. Skinnider 19. Immunoglobulin Light-Chain Amyloidosis . . . . . . . . . . . . . . . . 291
Morie A. Gertz, Taimur Sher, Angela Dispenzieri, and
8. Hodgkin Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115 Francis K. Buadi
Oliver W. Press and John P. Leonard
20. Macroglobulinemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303
9. Diffuse Large B-Cell Lymphoma and Related Neoplasms . . . . . 137 Steven P. Treon, Jorge J. Castillo, Zachary R. Hunter, and
Stephen D. Smith and Oliver W. Press Giampaolo Merlini
10. Follicular Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 21. Heavy-Chain Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 321
Oliver W. Press and John P. Leonard Dietlind L. Wahner-Roedler and Robert A. Kyle
11. Mantle Cell Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165 Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 331
Martin Dreyling
CONTRIBUTORS
Kenneth Anderson, MD Angela Dispenzieri, MD
Dana-Farber Cancer Institute Division of Hematology
Boston, Massachusetts Mayo Clinic
Rochester, Minnesota
Farrukh T. Awan, MD
Associate Professor of Internal Medicine Martin Dreyling, MD
Division of Hematology Department of Internal Medicine III
Department of Internal Medicine Medical Center of the University of Munich
The Ohio State University Comprehensive Cancer Center Munich, Germany
Columbus, Ohio
Andrew G. Evans, MD, PhD
Robert A. Baiocchi, MD, PhD Assistant Professor
Associate Professor of Medicine Department of Pathology and Laboratory Medicine
Division of Hematology University of Rochester Medical Center
Department of Internal Medicine Rochester, New York
The Ohio State University
Columbus, Ohio Aharon G. Freud, MD, PhD
Assistant Professor
Alessandro Broccoli, MD Department of Pathology
Institute of Hematology “L. e A. Seràgnoli” The Ohio State University
University of Bologna Columbus, Ohio
Bologna, Italy
Jonathan W. Friedberg, MD
Francis K. Buadi, MD Samuel Durand Professor of Medicine
Division of Hematology Director, Wilmot Cancer Institute
Mayo Clinic University of Rochester Medical Center
Rochester, Minnesota Rochester, New York
Francesca Cottini, MD
Dana-Farber Cancer Institute
Boston, Massachusetts
PREFACE
Bifurcation is an essential feature of biology. It underlies differentiation The lymphoid neoplasms are the subject of this text. Neoplasms
as one cell, through a process of mitosis accompanied by altered gene originating in the lymphoid progenitor cell hierarchy constitute the
expression, forms two distinct cell lineages. The hematopoietic system lymphomas and lymphocytic leukemias. These tumors afflicted over
is a dramatic example of this phenomenon. A single lymphohematopoi- 105,000 Americans and resulted in over 23,000 deaths in 2017. Their
etic stem cell, can over the course of several bifurcations, differentiate effects worldwide are dramatically larger. It is these compelling numbers
and then mature into at least 11 unique functional cells. In some cases, that prompted the editors to prepare a “breakaway” text on the malig-
these cells can mature further into different phenotypes influenced by nant lymphocytic neoplasms, based on the chapters that discussed these
the environment in which they reside. Consider, for example, the mono- diseases in the ninth edition of Williams Hematology. Approximately
cytes, Kupffer cells, osteoclasts, microglia, and alveolar macrophages. 3 years have passed since those chapters were written. The editors asked
One of the critical points of hematopoietic bifurcation is the dif- the authors of these 21 chapters to revise and update them in the light
ferentiation of the lymphohematopoietic stem cell into the common of three recent developments: an expanded classification of the lym-
myeloid and common lymphoid progenitor. It is at this point that phocytic neoplasms by the World Health Organization, advances in the
differentiation into these distinct lineages separates hematology into understanding of biology and genetics of these tumors, and advances in
two specialized areas of research and clinical practice: the myeloid and therapeutic approaches to the lymphomas and lymphocytic leukemias.
lymphoid neoplasms. Unlike most of the maturing myeloid cells, the The authors have graciously and expeditiously done so. With their help
lymphoid cells do not lose their mitotic capability. This requirement and expertise, we can now provide a timely text that covers the lymphomas
for continued replication and repair of DNA, along with the rearrange- and lymphocytic leukemias.
ments required of immunoglobulin and T-cell receptor genes dur- It is hoped the reader, from the accessibility of these new versions
ing maturation, provides the risk of neoplastic gene mutations; these of the chapters, will derive benefit in their research, clinical practice,
requirements result in a panoply of lymphocytic neoplasms, grossly and learning.
divided into B-lymphocyte, T-lymphocyte, and natural killer cell tumors.
The complexity of this array is extensive, with over 70 specific lympho- Marshall A. Lichtman
cytic tumors in the 2016 World Health Organization classification of Oliver W. Press
lymphocytic malignancies. John P. Leonard
: ~
MALIGNANT LYMPHOID tive work in this field, the International Lymphoma Study Group pro-
posed a classification termed the revised European-American Lymphoma
(REAL) classification (Chap. 6), 2 which was modified in 2001 and again
DISORDERS in 2008 by the World Health Organization (WHO). 3.4 The REAL/WHO
classification scheme makes use of the pathologic, immunophenotypic,
genetic, and clinical features of given lymphocyte tumors to delineate
Robert A. Baiocchi them into separate disease entities (Table 1-1 and Chap. 7). 5 For some of
these entities, the neoplastic lymphocytes have distinctive cytogenetic
abnormalities, which can be identified using molecular techniques that
are increasingly being used in clinical pathology laboratories. 6 •7
SUMMARY The REAL/WHO classification recognizes a basic distinction
between nodular lymphocyte-predominant Hodgkin lymphoma and
This chapter outlines the category of preneoplastic and neoplastic lymphocyte classic Hodgkin lymphoma, reflecting the differences in clinical presen-
and plasma cell disorders. It introduces a framework for evaluating neoplastic tation and behavior, morphology, phenotype, and molecular features
(Chap. 8). 3 Studies have identified features that can be used to distin-
lymphocyte and plasma cell disorders, outlines clinical syndromes associated
guish classical Hodgkin lymphoma from anaplastic large cell lymphoma
with such disorders, and guides the reader to the chapters in the text that
and, to a lesser extent, between nodular lymphocyte-predominant
discuss each of these disorders in greater detail. Hodgkin lymphoma and T-cell/histiocyte-rich large B-cell lymphoma.
The updated WHO classification (summarized in Ref. 4) provided
several revised guidelines for defining diseases such as chronic lym-
phocytic leukemia (CLL), 8 Waldenstrom macroglobulinemia, 9 plasma
e CLASSIFICATION cell neoplasms, 10 and diffuse large B-cell lymphoma (DLBCL). 11 - 14 The
classifications of several T-cell lymphomas were also refined, including
Lymphocyte and plasma cell malignancies present a broad spectrum enteropathy-associated T-cell lymphoma, anaplastic large cell lymphoma
of different morphologic features and clinical syndromes (Table 1-1). (ALK positive and ALK negative), and subcutaneous panniculitis-like
Lymphocyte neoplasms can originate from cells that are at a stage prior T-cell lymphoma.4 In 2014, a Clinical Advisory Committee meeting was
to T- and B-lymphocyte differentiation from a primitive stem cell or held to review literature and provide an update prior to the preparation
from cells at stages of maturation after stem cell differentiation. For of the next WHO tumor monograph series. The update reviews major
example, acute lymphoblastic leukemias arise from an early lymphoid areas from the WHO 2018 edition that changed significantly14" and are
progenitor cell that may give rise to cells with either B- or T-cell phe- summarized in Table 1-1.
notypes (Chap. 2), whereas chronic lymphocytic leukemia arises from
a more mature B-lymphocyte progenitor (Chap. 3) and myeloma from
progenitors at even later stages ofB-lymphocyte maturation (Chap. 18).
Disorders oflymphoid progenitors may result in a broad spectrum oflym-
. CLINICAL BEHAVIOR
phocytic diseases, such as B- orT-celllymphomas (Chaps. 9 and 15), hairy Lymphomas of similar histology can have widely different spectra of
cell leukemia (Chap. 4), prolymphocytic leukemia (Chap. 3), natural associated clinical symptoms and clinical aggressiveness, making the
categorization of lymphoid tumors impossible using a generic grad-
ing system based on morphology alone. For example, the neoplastic
cells in mantle cell lymphoma appear smaller and more differentiated
Acronyms and Abbreviations: a/PTCR, T-cell-receptor genes encoding the a and p than those of anaplastic large cell lymphomas. However, the validation
chains of the T-cell receptor; ALK, gene encoding anaplastic lymphoma kinase; BCL2, studies for the REAL classification revealed that patients with mantle
gene encoding B-cell chronic lymphocytic leukemia (CLL)/lymphoma 2; BCL6, gene cell lymphoma and anaplastic large cell lymphomas have 5-year sur-
encoding B-cell chronic lymphocytic leukemia (CLL)/lymphoma 6; clg, cytoplasmic vival rates of approximately 30 percent and approximately 80 percent,
immunoglobulin; EBER, Epstein-Barr-virus-encoded RNA; EBV, Epstein-Barr virus; respectively. 15• 16 Generally, T-cell lymphomas/leukemias have a more
y/oTCR, T-cell-receptor genes encoding they and ochains of the T-cell receptor; HL, aggressive clinical behavior than B-cell lymphomas of comparable his-
Hodgkin lymphoma; HLA, human leukocyte antigen; HTLV-1, human T-cell leukemia tology. The tendency for more aggressive disease also applies to lym-
virus type 1; HHV8, human herpes virus 8; lg, immunoglobulin; lgR, immunoglobulin phoid tumors derived from natural killer cells. A helpful distinction
gene rearrangement; IL, interleukin; MALT, mucosa-associated lymphoid tissue; is to divide the lymphoid tumors into one of two categories, namely,
MUM/, gene encoding multiple myeloma oncogene 1; neg., negative; NK cell, natural indolent lymphomas versus aggressive lymphomas, based upon on the
killer cell; NOS, not otherwise specified; NPM, gene encoding nucleophosmin; PAXS, characteristics of the disease at the time of presentation and patients'
paired box gene S; POEMS, polyneuropathy, organomegaly, endocrinopathy, mono- life expectancy if the disease is left untreated. 17 •18 Clinical studies have
clonal gammopathy, and skin changes; REAL, revised European-American lymphoma; verified that the different disease categories defined in the REAL/WHO
R-S, Reed-Sternberg; slg, surface immunoglobulin; slgD, surface immunoglobulin D; classification each can be segregated into one or the other of these two
slgM, surface immunoglobulin M; TAL 1, gene encoding T-cell acute leukemia-1; major categories (Tables 1- 2 and 1- 3, respectively). 15 Analyses of gene-
TCR, T-cell receptor; TdT, terminal deoxynucleotidyl transferase; Th 2, T-helper type 2; expression patterns using microarray technology have enabled identi-
WHO, World Health Organization. fication of subcategories within some of the disease categories defined
by the REAL/WHO classification that have different tendencies for
2 Williams Hematology Malignant Lymphoid Diseases
TABLE 1–1. Classification of Lymphoma and Lymphoid Leukemia by the World Health Organization
Neoplasm Morphology Phenotype* Genotype†
B-CELL NEOPLASMS
Immature B-Cell Neoplasms
Lymphoblastic leukemia/ Medium-to-large cells with TdT+, sIg–, CD10+, CD13+/–, CD19+, Clonal DJ rearrangement of IGH gene
lymphoma not otherwise finely stippled chromatin CD20–, CD22+, CD24+, CD34+/–, T(17;19), E2A-HLF, AML1 iAMP21 asso-
specified (NOS) (Chap. 2) and scant cytoplasm CD33+/–, CD45+/–, CD79a+, PAX5+ ciated with poor prognosis
Lymphoblastic leukemia/ See above See above. B-ALL with t(9;22) with See individual genetic features in
lymphoma with recurrent CD25 and more frequent myeloid B-ALL subtypes below
genetic abnormalities antigens CD13, CD33
(Chap. 2)
B
-ALL with t(v;11q23); See above CD19+, CD10–, CD24–, CD15+ Multiple MLL (11q23) fusion partners,
MLL rearranged including AF4 (4q21), AF9 (9p22),
and ENL (19p13). B-ALL with MLL
translocations overexpress FLT-3.
Poor prognosis
B
-ALL with t(12;21) See above CD19+, CD10+, CD34+. Characteris- t(12;21)(p13;q22) ETV6-RUNX
(p13;q22); TEL-AML1 tically negative for CD9, CD20, and translocation
(ETV6-RUNX1) CD66c
B
-ALL with See above CD19+, CD10+, CD45–, CD34+ Numerical increase in chromosomes
hyperdiploidy without structural abnormalities.
Most frequent chromosomes +21,
X, 14, and 4. +1, 2, 3 rarely seen.
Favorable prognosis
B
-ALL with See above See above Loss of at least one or more chromo-
hypodiploidy somes (range from 45 chromosomes
to near haploid). Rare chromosome
abnormalities. Poor prognosis
B
-ALL with t(5;14) See above with increase in See above. Even rare blasts with B-ALL t(5;14)(q31;q32); IL3-IGH leading
(q31;q32); IL3-IGH reactive eosinophilia immunophenotype with eosinophilia to overexpression of IL3. Unclear
strongly suggestive of this subtype of prognosis
B-ALL
B
-ALL with t(1;19) See above CD10+, CD19+, cytoplasmic μ heavy t(1;19)(q23;p13.3); leads to overex-
(q23;p13.3); E2A-PBX1 chain. CD9+, CD34– pression of E2A-PBX1 fusion gene
product interfering with normal
transcription factor activity of E2A
and PBX1
Mature B-Cell Neoplasms
Leukemias
Chronic lymphocytic Small cells with round, sIg+(dim), CD5+, CD10–, CD19+, IgR+, trisomy 12 (~30%), del at 13q14
leukemia/small lym- dense nuclei CD20+(dim), CD22+(dim), CD23+, (~50%), 11q22–23, 17p13, and IGHV
phocytic lymphoma CD38+/–, CD45+, FMC-7– mutated status associated with
(Chap. 3) poor prognosis. Mutations in TP53,
NOTCH1, SF3B1, ATM, and BIRC3
Prolymphocytic leuke- ≥55% prolymphocytes sIg+(bright), CD5+/–, CD10–, CD19+, del13q.14(~30%); del17p (50%), IgR+
mia (Chap. 3) CD22+, CD23+/–, CD45+, CD79a+,
FMC7+
Hairy cell leukemia Small cells with cytoplas- sIg+(bright), CD5–, CD10–, BRAF mutations (~100%), IgR+ MAP2K
(Chap. 4) mic projections CD11c+(bright), CD19+, CD20+, mutations in BRAF wt
CD25+, CD45+, CD103+, Annexin A+
Lymphomas
Lymphoplasmacytic Small cells with plasmacy- cIg+, CD5–, CD10–, CD19+, CD20+/– IgR, 6q- in 50% of marrow-based
lymphoma (Chap. 20) toid differentiation Plasma cell population: CD38+, cases [the t(9;14) was proved to be
CD138+, cIgM+ wrong], +4 (20%)
(Continued )
“Rosemary, my dear, I wish you would not dance all the time with
young Roland Bayard when you happen to be at a party with him,”
said the grave and dignified Miss Susannah Grandiere to the fair
little niece who sat at her feet, both literally and figuratively.
The early tea was over at Grove Hill, and the aunt and niece sat
before the fire, with their maid Henny in attendance.
Miss Grandiere was knitting a fine white lamb’s wool stocking;
Rosemary was sewing together pieces for a patchwork quilt; and
Henny, seated on a three-legged stool in the chimney corner, was
carding wool.
“Why not, Aunt Sukey?” inquired the child, pushing the fine, silky
black curls from her dainty forehead and looking up from her work.
“Because, my dear, though you are but a little girl, and he is almost
a young man, yet these intimate friendships, formed in early youth,
may become very embarrassing in later years,” gravely answered the
lady, drawing out her knitting needle from the last taken off stitch
and beginning another round.
“But how, Aunt Sukey?” questioned the little one.
“In this way. No one knows who Roland Bayard is! He was cast up
from the wreck of the Carrier Pigeon, the only life saved. He was
adopted and reared by Miss Sibby Bayard, and I think, but am not
sure, he was educated at the expense of Abel Force, who never lets
his left hand know what his right hand does in the way of charity. But
Miss Sibby has hinted as much to me.”
“Aunt Sukey, he may be the son of a lord, or a duke, or a prince,”
suggested romantic Rosemary.
“Or of a thief, or pirate, or convict,” added Miss Grandiere,
severely.
“Oh, Aunt Sukey! Never! Never! Dear Roland! Aunt Sukey, I like
Roland so much! And I have good reason to like him, too, whatever
he may be!” exclaimed the child, with more than usual earnestness.
“Oh! oh! oh!” moaned Miss Grandiere, sadly, shaking her head.
“Aunt Sukey, no one ever has the kindness to ask a little girl like
me to dance except dear Roland. Other gentlemen ask young ladies;
but dear Roland always asks me, and he never lets me be neglected.
And I shall never forget him for it, but shall always like him.”
“Um, um, um!” softly moaned the stately lady to herself.
“And Roland told me he was named after a knight who was
‘without fear and without reproach,’ and that he meant always to
deserve his name, and to be my knight—mine.”
“Dear, dear, dear!” murmured Miss Grandiere.
“What is the matter, Aunt Sukey?” inquired Rosemary, again
pushing back her silky, black curls, and lifting her large, light blue
eyes to the lady’s troubled face.
“Rosemary, my child,” began Miss Grandiere, with out replying to
the little girl’s question, “Rosemary, you know the Forces are going to
Washington next week?”
“Oh! yes; everybody knows that now.”
“And Wynnette and Elva are going to be put to school there?”
“Yes, everybody knows that, too, Aunt Sukey.”
“Well, how would you like to be put to the same school that they
are going to attend?”
“Oh, so much! So very much, Aunt Sukey! I never dreamed of such
happiness as that! I do so much want to get a good education!”
exclaimed the little girl, firing with enthusiasm.
“Well, my dear child, I think the opportunity of sending you to
school with Wynnette and Elva, and under the protection of Mr. and
Mrs. Force, is such an excellent one that it ought not to be lost. I will
speak to my sister Hedge about it, and if she will consent to your
going I will be at the cost of sending you,” said the lady, as she began
to roll up her knitting, for the last gleam of the winter twilight had
faded out of the sky and it was getting too dark even to knit.
For once in her life Rosemary had forgotten to call for the curtains
to be let down and the candle to be lit and the novel brought forth.
For once the interests of real life had banished the memory of
romance.
But Henny knew what was expected of her, and so she put up her
cards, went and lighted the tallow candle, pulled down the window
blinds, replenished the fire, and reseated herself on her three-legged
stool in the chimney corner.
Rosemary, recalled to the interests of the evening, went and
brought forth the “treasured volume” from the upper bureau drawer
and gave it to her aunt to read. Then she settled herself in her low
chair to listen.
It was still that long romance of “The Children of the Abbey” that
was the subject of their evening readings. And they had now reached
a most thrilling crisis, where the heroine was in the haunted castle;
when suddenly the sound of wheels was heard to grate on the gravel
outside, accompanied by girlish voices.
And soon there came a knock at the door.
“Who in the world can that be at this hour, after dark?” inquired
Miss Grandiere, as Henny arose and opened the door.
Odalite, Wynnette and Elva came in, in their poke bonnets and
buttoned coats.
“Oh, Miss Grandiere, excuse us, but yours was the only light we
saw gleaming around the edges of the blinds, and so we knocked at
your door,” said Wynnette, who always took the initiative in
speaking, as in other things.
“My dear child! how is it that you children are out, after dark?”
inquired the lady.
“We have been making the rounds to bid good-by to the neighbors.
Mamma and papa went out yesterday, and we to-day. We are going
to Washington next week, and we have come to bid you good-by
now,” said Wynnette, still speaking for all the others.
“But who is with you for protection? Who drove the carriage?”
“Jake drove and Joshua came as bodyguard; but we are so late that
I am afraid Mr. and Mrs. Elk and the girls are asleep.”
“They are, my dears; and it is so late that I do not think it right for
you three children to be driving through the country with no better
protection than Jake and the dog. You must send them home and
stay all night here. Then you will have an opportunity of bidding
good-by to William and Molly and the children to-morrow morning.”
“Oh, Miss Grandiere, how jolly! I have not spent a night from
home for ages and ages and ages!” exclaimed Wynnette.
“But what will mamma say?” doubtfully inquired Elva.
“I fear, Miss Grandiere, that we ought to return home to-night,”
suggested Odalite.
“Nonsense, my dear child! You must do nothing of the sort. I will
write a note to your mother and send it by Jake,” replied Miss
Grandiere, who immediately arose and went to get her portfolio.
“If it hadn’t been for Miss Sibby Bayard keeping us so long talking
about her ancestor the ‘Duke of England’—she means the Duke of
Norfolk all the time, but flouts us when we hint as much—we should
have been here two hours ago, and been home by this time,” said
Wynnette.
Miss Grandiere finished her note, put a shawl over her head and
went out herself to speak to the coachman and send him home to
Mondreer with her written message.
“Now take off your hats and coats, and tell me if you have had tea,”
she said, when she came back into the room and closed the door.
“Oh, yes! we took tea with Miss Sibby while she told us how a
certain ‘Duke of England’ lost his head for wanting to marry a certain
Queen of Scotland,” replied Wynnette.
That question settled, the girls drew chairs around the fire, and
began to make themselves comfortable.
Rosemary could not bear to give up her reading, just at that
particular crisis, too! So she thought she would entice her company
into listening to the story.
“We were reading—oh! such a beautiful book!” she said. “Just hear
how lovely it begins!”
And she took the book up, turned it to the first page and
commenced after this manner:
“‘Hail! sweet asylum of my infancy! Content and innocence abide
beneath your humble roof!—hail! ye venerable trees! My happiest
hours of childish gayety——’”
“What’s all that about?” demanded Wynnette, the vandal,
ruthlessly interrupting the reader.
“It is Amanda Fitzallan, coming back to the Welsh cottage where
she was nursed, and catching sight of it, you know, raises fluttering
emotions in her sensitive bosom,” Rosemary explained, with an
injured air.
“Oh! it does, does it? But she wouldn’t hold forth in that way, you
know, even if she were badly stage struck or very crazy,” said
Wynnette.
“Oh! I thought it was such elegant language!” pleaded Rosemary.
“But she wouldn’t use it! Look here! Do you suppose, when I come
back from school, years hence, and catch sight of Mondreer, I should
hold forth in that hifaluting style?”
“But what would you say?”
“Nothing, probably; or if I did, it would be: ‘There’s the blessed old
barn now, looking as dull and humdrum as it did when we used to go
blackberrying and get our ankles full of chego bites. Lord! how many
dull days we have passed in that dreary old jail, especially in rainy
weather!’ I think that would be about my talk.”
“Oh, Wynnette! you have no sentiment, no reverence, no——”
“Nonsense!” good-humoredly replied the girl, finishing
Rosemary’s halting sentence.
The little girl sighed, closed the book and laid it on the table.
“The style of that work is very elegant and refined; and it is better
to err on the side of elegance and refinement than on their
opposites,” said Miss Grandiere, with her grandest air.
“As I do every time I open my mouth. But I can’t help it, Miss
Susannah. ‘I am as Heaven made me,’ as somebody or other said—or
ought to have said, if they didn’t,” retorted Wynnette.
As it was now bedtime it became necessary to attend to the
sleeping accommodations of these unexpected guests. But first it was
in order to offer them some refreshments. Henny was not required to
draw a jug of hard cider, or to make and bake hoe cakes in the
bedroom that night. Such “orgies” were only enacted by the aunt and
niece in the seclusion of their private life.
But the corner cupboard was unlocked, and a store of rich cake
and pound cake, with a cut-glass decanter of cherry bounce, all of
which was kept for company, was brought forth and served to the
visitors.
Meanwhile, Henny went upstairs to kindle a fire in the large,
double-bedded spare room, just over Miss Sukey’s chamber.
“Miss Susannah,” said Odalite, while the group sat around the fire
nibbling their cake and sipping their bounce, “I have a favor to ask of
you.”
“Anything in the world that I can do for you, Odalite, shall be done
with the greatest pleasure,” earnestly replied the elder lady.
“I thank you very much, dear friend; and now I will explain: I
promised Le, before we went away, that I would go to Greenbushes
once a week to see that the rooms were regularly opened, aired and
dried. I have kept the promise up to the present; but now, you know,
I have to go with the family to Washington. I have no alternative, and
for that reason I would like you to be my proxy.”
“I will, with great pleasure, my dear.”
“I could not ask you to go every week, that would be too much; but
if you can go occasionally and see that all is right, and drop me a note
to that effect, it will—well, it will relieve my conscience,” concluded
Odalite, with a wan smile.
“I certainly will go every week, unless prevented by circumstances;
and I will write to you as often as I go, to let you know how all is
getting on there.”
“Oh, you are very kind, Miss Susannah; but I fear you will find it a
tax upon your time and patience.”
“Not at all. I shall have plenty of time, and little that is interesting
to fill it up with. For let me tell you a secret. I intend to avail myself
of the opportunity of your parents being in Washington to send my
little Rosemary to the same school that Wynnette and Elva will
attend.”
“Oh, that will be jolly!” “Oh, that will be lovely!” exclaimed
Wynnette and Elva, in the same instant.
“That is, if Mr. and Mrs. Force will not consider the addition of
Rosemary to their party an intrusion.”
“Why, Miss Susannah! How dare you slander my father and
mother right before my two looking eyes?” exclaimed Wynnette.
“They will be just set up to have Rosemary! Besides, where’s the
intrusion, I’d like to know? The railroad and the hotel and the
boarding school are just as free for you as for me, I should think.”
“Rosemary would board at the school, of course,” continued Miss
Grandiere.
“So shall Elva and I. If papa could have got a furnished house we
should have lived at home, and entered the academy as day pupils;
but, you see, as papa could not get a house he and mamma and
Odalite will live at one of the West End hotels, and Elva and I at the
academy.”
“And, oh! won’t it be lovely to have dear Rosemary with us? We
should not feel half so strange,” said little Elva.
“You will speak to your father and mother on the subject when you
go home, Odalite, my child; and I will call on them later. If they will
take charge of Rosemary on the journey, and enter her at the same
school with yourselves, I will be at all the charges, of course, and I
shall feel very much obliged,” said Miss Susannah.
“You may rest assured that papa and mamma will be very glad to
take charge of dear little Rosemary; not only for her sake and for
your sake, but for our sakes, so that we may have an old playmate
from our own neighborhood to be our schoolmate in the new home,”
said Wynnette.
“There is something in that,” remarked Miss Grandiere.
As for Elva and Rosemary, they were sitting close together on one
chair, with their arms locked around each other’s waist, in fond
anticipation of their coming intimacy.
Henny now came in and said that the spare room was all ready for
the young ladies.
Miss Grandiere lighted a fresh candle, and conducted her visitors
to the upper chamber, saw that all their wants were supplied, and
bade them good-night.
Soon after, aunt and niece also retired to bed; but Rosemary could
not sleep for the happiness of thinking about going to boarding
school in the city along with Wynnette and Elva.
Early in the morning William and Molly Elk, their little girls, and
in fact the whole household, with the exception of Miss Sukey, her
niece and her maid, were astonished to hear that there were visitors
in the house who had arrived late on the night before.
They prepared a better breakfast than usual in their honor, and
gave them a warm welcome.
Soon after breakfast, Jake arrived with the family carriage to fetch
the young people home, and also with a message from Mr. and Mrs.
Force, thanking Miss Grandiere for having detained their imprudent
children all night.
“You and Rosemary go home with us, Miss Susannah. There’s
plenty of room inside the carriage for six people, and we would only
be five. Do, now! And let us have this matter of going to school
settled at once,” urged Wynnette.
Miss Grandiere hesitated, even though Elva joined in the
invitation. But when Odalite, the eldest and grown-up sister, added
her entreaties to those of the others, Miss Sukey yielded, because she
wanted to yield.
The girls then took leave of all their friends at Grove Hill and
entered the capacious carriage, accompanied by Miss Grandiere and
Rosemary—that is, two of them did. One was missing.
“Where is Wynnette?” inquired Miss Grandiere, as she sank into
the cushions.
“She is on the box, driving, while Jacob is sitting with folded arms
beside her,” answered Odalite.
“It is highly improper.”
“You cannot do anything with Wynnette, Miss Susannah. She will
drive as often as she can. And Jacob’s presence beside her makes it
safe, at least. He is ready to seize the reins at any emergency.”
“Yes, but really—really—my dear Odalite——”
The sudden starting of the horses at a spanking pace jerked Miss
Grandiere’s words from her lips, and herself forward into little Elva’s
arms.
However, they arrived safely at Mondreer, where they were very
cordially welcomed by Mr. and Mrs. Force.
When Miss Grandiere proposed her plan of sending Rosemary
with them, to go to school with their own children, the lady and
gentleman responded promptly and cordially.
“We have not selected our school yet,” Mr. Force explained. “We
wish to get the circulars and personally inspect the schools before we
make our choice, but if you leave your niece in our hands, we shall do
by her exactly as by our own.”
“I am sure you will. And I thank you from my soul for the trouble
you take. I shall sign some blank checks, for you to fill out, for any
funds that may be required for Rosemary,” gratefully responded Miss
Grandiere.
The aunt and niece, at the cordial invitation of the Forces, stayed
to dinner, and were afterward sent home in a wide buggy driven by
Jacob.
One day later Miss Grandiere broached to Mrs. Hedge the subject
of sending Rosemary to school with Wynnette and Elva Force, at her
own—Miss Grandiere’s—expense.
This consultation with the mother was a mere form, Miss
Susannah knowing full well that it was the great ambition of Mistress
Dolly’s heart to send her daughter to a good boarding school, and
that she would consider the present opportunity most providential.
All the arrangements were most satisfactorily concluded, and by
the end of the following week, the Forces, with little Rosemary in
their charge, had left Mondreer.
CHAPTER IV
AFTER A LAPSE OF TIME
It was three years after the Forces left Mondreer, and they had
never returned to it.
The farm was managed by Jesse Barnes, the capable overseer, and
the sales were arranged by Mr. Copp, the family agent, who remitted
the revenues of the estate in quarterly installments to Mr. Force.
The lady from the gold mines remained in the house, taking such
excellent care of the rooms and the furniture that she had gradually
settled down as a permanent inmate, in the character of a salaried
housekeeper.
“I’m a-getting too old to be bouncing round prospecting with the
boys, and so I reckon I had better sit down in this comfortable
sitiwation for the rest of my life,” she confided to Miss Bayard, one
February morning, when that descendant of the great duke honored
her by coming to spend the day at Mondreer.
“That’s just what I sez myself. When you knows you’re well enough
off, sez I, you’d better let well enough alone, sez I. And not take after
them unsettled people as are allus changing about from place to
place, doing no good,” assented Miss Bayard.
“It’s a habit dey gibs deirselves. ’Deed it is, ole mist’ess. Nuffin’ ’t
all but a habit dey gibs deirselves,” remarked Luce, who had just
come in with a waiter, on which was a plate of caraway-seed cake and
a decanter of blackberry cordial to refresh the visitor.
“Just like my neffy, Roland. He was restless enough after Le went
to sea, but after the Forces left the neighborhood and took Rosemary
Hedge with ’em, ropes nor chains wouldn’t hold that feller, but he
must go off to Baltimore to get a berth, as he called it. Thanks be to
goodness, he got in ’long of Capt. Grandiere as first mate; but Lord
knows when I’ll ever see him ag’in, for he is gone to the East Indies,”
sighed Miss Sibby. And then she stopped to nibble her seed cake and
sip her blackberry cordial.
“It’s a habit he gibs hisself, ole mist’ess. ’Deed it is. Nuffin’ ’t all but
a habit he gibs hisself, and you ought to try to break him of it,” said
Luce, as she set the waiter down on the table and left the room.
“Do you expect Abel Force ever to come home to his own house
again?” inquired Miss Sibby, between her sips and nibbles.
“Oh, yes, I reckon so, when the gals have finished their edication,
but not till then. You see they have a lovely house in Washington,
according to what Miss Grandiere and little Rosemary Hedge tells us,
and the children are at a fine school, so they live there all the year
until the three months summer vacation comes round, and then
when Miss Grandiere goes to Washington to fetch her little niece
home to spend the holidays here, why, then Mr. and Mrs. Force takes
their three daughters and go traveling. And this next summer they do
talk about going to Europe, but I don’t know that they will do it.”
“What I sez is that they ought to spend their summers at
Mondreer. When a family is blessed with the blessing of a good,
healthy country home, sez I, they ought to stay in it, and be thankful
for it, sez I.”
Even while the two cronies spoke the door opened, and Jacob
came in, with a letter in his hand.
“There! That’s from the ole ’oman now. I know her handwriting
across the room. And now we shall hear some news,” said Mrs.
Anglesea, with her mouth full of cake.
And she took the letter from the negro’s hands, and opened it
without ceremony, and began to read it to herself, without apology.
“Is it anything confidential?” demanded Miss Sibby, who was full
of curiosity.
“No. I will read it all to you as soon as ever I have spelled it out
myself. I never was good at reading writing, particularly fine hand,
and, if I must say it, the ole ’oman do write the scrimble-scramblest
fine hand as ever I see,” said Mrs. Anglesea, peering at the letter, and
turning it this way and that, and almost upside down.
Presently she began to read, making comments between the words
and phrases of the letter.
“Well, it’s ‘Washington City, P Street, N. W., and February 8th.’
Why, it’s been four days coming. Here you, Jake! When did you get
this letter out’n the post office?” She paused to call the negro
messenger, who stood, hat in hand, at the door.
“W’y, dis mornin’, in course, ole mist’ess,” replied the man.
“Don’t ‘ole mist’ess’ me, you scalawag! Are you sartain you didn’t
get it Saturday, and forget all about it, and leave it in your pocket
until to-day?”
“Hi, ole—young—mist’ess, how I gwine to forget w’en you always
ax me? No, ’deed. I took it out’n de pos’ office dis blessed mornin’,
ole—young mist’ess.”
“How dare you call me young mist’ess, you——”
“What mus’ I call you, den?” inquired the puzzled negro.
“Ma’am. Call me ma’am.”
“Yes, ma’am.”
“That’s better. Well, now the next time you go to the village, Jake,
you just tell that postmaster if he keeps back another letter of mine
four days, I’ll have him turned out. Do ye hear?”
“Yes, ma’am.”
“Well, now you may go about your business, and I will go on with
my letter.”
“Yes, ma’am.”
The man left the room, and the housekeeper resumed her reading:
“‘My Dear Mrs. Anglesea’: I wish she wouldn’t pile that name upon me so! If
she knowed how I hated it she wouldn’t. ‘I write to ask you to have the house
prepared for our reception on the eighth of June. You will know what is necessary
to be done, and you may draw on Mr. Copp for the needful funds. He has
instructions to honor your drafts.
“‘The girls expect to grad—grat—gral—gual——’
“Lord ’a’ mercy! what is this word? Can you make it out, Miss
Sibby?” inquired the reader, holding the letter under the nose of the
visitor.
Miss Bayard, who had resumed her knitting after moderately
partaking of cake and cordial, dropped her work, adjusted her
spectacles and inspected the word.
“It’s graduate, ma’am. That means finish their edication,
honorable. Young Le Force graduated offen the Naval ’Cademy
before he ever went to sea as a midshipman, and my scamp, Roland
Bayard, graduated offen the Charlotte Hall ’Cademy before he ran
away and went to sea as a common sailor. I s’pose these girls is a-
going to graduate offen the ‘cademy where they are getting their
edication, and I hope they will do theirselves credit. When your
parents do the best they can for you, sez I, you ought to try to do the
best you can for yourself, sez I, which is the best return you can make
them, sez I.”
“To do the best you can for them, I should think would be the first
thing to think about, and, likewise, best return to make them. But
now I’ll go on with my letter:
“‘The girls expect to graduate at the academic commencement, on the first of
June’—graduate at the commencement! I thought pupils graduated at the end!
—‘after which we expect to come down to Mondreer for the summer, previous to
going to Europe. I have much news of importance to tell you, which concerns
yourself as much as it affects us; but it is of such a nature that it had best be
reserved for the present. Expecting to see you, I remain your friend,
Elfrida Force.’”
“So they are actually coming home at last,” said Miss Sibby.
“Yes, actially coming home at last,” assented the housekeeper.
“But, look here. What does she mean by that news as she has got to
tell me which concerns she and I both? I reckon it must be news of
my rascal. Lord! I wonder if it is? I wonder if he’s been hung or
anything? I hope to gracious he has! And then she wouldn’t mention
it in a letter, but wait until she could tell me all about it! It must be
that, ole ’oman—my rascal’s hung!”
“I reckon it is! When a man lives a bad life, sez I, he must expect to
die a bad death, sez I.”
“Well, I shan’t go in mourning for him, that’s certain, whether he’s
hung or drowned. But we shall hear all about it when the folks come
home. Lord! why, the place will be like another house, with all them
young gals in it!”
“I might ’a’ knowed somethin’ was up t’other Sunday, when I heard
Miss Grandiere tell Parson Peters, at All Faith Church, how she and
Mrs. Hedge were both going to Washington on the first of June. Of
course, it is to the commencement they’re going, to see Rosemary
graduate along with the others.”
“But to hear ’em call the end of a thing its commencement, takes
me,” said Mrs. Anglesea.
“So it do me. And if people don’t know what they’re a-talking
about, sez I, they’d better hold their tongues, sez I.”
“Young Mrs. Ingle will be mighty proud to have the old folks and
the gals back. Lord! how fond she was of them two little gals. To
think of her naming her two babies after them—the first Wynnette
and the second Elva. Let’s see; the first one must be two years old.”
“Wynnie is twenty-three months old, and Ellie is nine months; but
they are both sich smart, lively, sensible children that any one might
think as they was older than that. But I don’t hold with children
being took so much notice of, and stimmerlated in their intellects so
much. Fair an’ easy, sez I; slow and sure, sez I, goes a long way, sez
I.”
So, talking about their neighbors, as usual, but not uncharitably,
the gossips passed the day. At sunset they had tea together; and then
Gad brought around the mule cart—the only equipage owned by the
descendant of the great duke—who put on her bonnet and shawl, bid
good-by to her crony, got into her seat and drove homeward.
“Well, the ole ’oman has give me long enough notice to get ready
for ’em; but she knows there’s a good deal to be done, and country
workmen is slow, let alone the niggers, who is slowest of all,”
ruminated Mrs. Anglesea, who resolved to begin operations next day.
CHAPTER V
THE FORTUNES OF ODALITE