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Williams Manual of Hematology, Ninth Edition Marshall A.
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Williams
Manual of Hematology
NOTICE
Medicine is an ever-changing science. As new research and clinical experience broaden our
knowledge, changes in treatment and drug therapy are required. The authors and the
publisher of this work have checked with sources believed to be reliable in their efforts to
provide information that is complete and generally in accord with the standards accepted at
the time of publication. However, in view of the possibility of human error or changes in
medical sciences, neither the authors nor the publisher nor any other party who has been
involved in the preparation or publication of this work warrants that the information
contained herein is in every respect accurate or complete, and they disclaim all
responsibility for any errors or omissions or for the results obtained from use of the
information contained in this work. Readers are encouraged to confirm the information
contained herein with other sources. For example and in particular, readers are advised to
check the product information sheet included in the package of each drug they plan to
administer to be certain that the information contained in this work is accurate and that
changes have not been made in the recommended dose or in the contraindications for
administration. This recommendation is of particular importance in connection with new or
infrequently used drugs.
Williams
Manual of Hematology
Ninth Edition
Marshall A. Lichtman, MD
Professor of Medicine (Hematology-Oncology)
and of Biochemistry and Biophysics
James P. Wilmot Cancer Institute
University of Rochester Medical Center
Rochester, New York
Kenneth Kaushansky, MD
Senior Vice President, Health Sciences
Dean, School of Medicine
SUNY Distinguished Professor
Stony Brook University
Stony Brook, New York
Josef T. Prchal, MD
Professor of Medicine, of Pathology, and of Genetics
Division of Hematology
University of Utah
Salt Lake City, Utah
First Faculty of Medicine
Charles University
Prague, Czech Republic
Linda J. Burns, MD
Professor of Medicine
Division of Hematology, Oncology, and Transplantation
University of Minnesota
Minneapolis, Minnesota
James O. Armitage, MD
The Joe Shapiro Professor of Medicine
Division of Oncology and Hematology
University of Nebraska Medical Center
Omaha, Nebraska
New York Chicago San Francisco Athens London Madrid Mexico City
Milan New Delhi Singapore Sydney Toronto
Williams Manual of Hematology, Ninth Edition
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Copyright Act of 1976, no part of this publication may be reproduced or distributed in any form or by an means, or stored in a data
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CONTENTS
Preface
48 Classification and Clinical Manifestations of Polyclonal Lymphocyte and Plasma Cell Disorders
49 Lymphocytosis and Lymphocytopenia
50 Primary Immunodeficiency Syndrome
51 Hematological Manifestations of the Acquired Immunodeficiency Syndrome
52 The Mononucleosis Syndromes
78 Hemophilia A and B
79 von Willebrand Disease
80 Hereditary Disorders of Fibrinogen
81 Inherited Deficiencies of Coagulation Factors II, V, V + VIII, VII, X, XI, and XIII
82 Antibody-Mediated Coagulation Factor Deficiencies
83 Hemostatic Dysfunction Related to Liver Diseases
84 The Antiphospholipid Syndrome
85 Disseminated Intravascular Coagulation
86 Fibrinolysis and Thrombolysis
Williams Manual of Hematology provides a convenient and easily navigable précis of the
epidemiology, etiology, pathogenesis, diagnostic criteria, differential diagnosis, and therapy of
blood cell and coagulation protein disorders. The 93 chapters in the Manual are a distillation of
the disease- and therapy-focused chapters of the ninth edition of Williams Hematology. The
Manual is a handbook, but it is comprehensive. It is organized into 12 parts, paralleling the ninth
edition of Williams Hematology, yet of a size that permits it to serve as a companion to the
physician in the hospital or clinic. It can be used as a hard copy carried in one’s coat pocket or,
more deftly, as an app on one’s smart phone or tablet.
We have included chapters on the classification of red cell, neutrophil, monocyte, lymphocyte,
and platelet disorders and of diseases of coagulation proteins to provide a framework for
considering the differential diagnosis of syndromes that are not readily apparent. Also included
are numerous tables that contain diagnostic and therapeutic information relevant to the diseases
discussed. Detailed chapters describing the features of individual myeloid and lymphoid
malignancies provide a guide to diagnosis, staging, and management. Chapters on the
manifestations, diagnostic criteria, and therapy of hereditary and acquired thrombophilia consider
the role hematologists play in diagnosing and managing this important mechanism of disease.
Descriptions of diseases of red cells, neutrophils, monocytes, macrophages, lymphocytes,
platelets, and coagulation proteins and their management leave no gaps and meet the needs of the
busy hematologist, internist, or pediatrician. In addition, this handbook is very useful for
advanced practice professionals, medical and pediatric residents and subspecialty fellows, and
medical or nursing students because of its succinct clinical focus on diagnosis and management.
For many tables reproduced in the Manual, the reader can find explicit citations documenting
those entries in the concordant chapter in the ninth edition of Williams Hematology. In addition,
where helpful, images of blood or marrow cell abnormalities or external manifestations of
disease are included. Each chapter ends with an acknowledgment of the authors of the relevant
chapter in the ninth edition of Williams Hematology, including the chapter title and number for
easy cross-reference to that comprehensive text.
The publisher prints a caution in the Manual that admonishes readers to verify drug doses,
routes of administration, timing of doses, and duration of administration and to check the
contraindications and adverse effects of drugs used to treat the diseases described. We
reemphasize that these often complex diseases require direct participation and close supervision
of an experienced diagnostician and therapist. This oversight should be provided by a person who
is able to individualize therapy depending on the nature of the expression of the primary
hematological disease, the patient’s physiological age, and the presence of coincidental medical
conditions, among other factors.
The authors acknowledge the valuable assistance of Marie Brito at Stony Brook University,
Kim Arnold at the University of Nebraska, and, notably, Susan Daley at the University of
Rochester, who entered tables and figures into the chapters, managed the administrative
requirements in the preparation of the Manual, and coordinated communication among the six of
us and McGraw-Hill. We also acknowledge the encouragement and support of Karen Edmonson,
Senior Content Acquisitions Editor, and Harriet Lebowitz, Senior Project Development Editor, at
the Medical Publishing Division, McGraw-Hill Education.
The care of a patient with a hematologic disorder begins with eliciting a medical history and
performing a thorough physical examination. Certain parts of the history and physical examination
that are of particular interest to the hematologist are presented here.
FAMILY HISTORY
Hematologic disorders may be inherited as autosomal dominant, autosomal recessive, or X
chromosome–linked traits (see Williams Hematology, 9th ed, Chap. 10). The family history is
crucial to provide initial clues to inherited disorders and should include information relevant
to the disease in question in grandparents, parents, siblings, children, maternal uncles and
aunts, and nephews. Careful and repeated questioning is often necessary because some
important details, such as the death of a sibling in infancy, may be forgotten years later.
Consanguinity should be considered in a patient who belongs to a population group prone to
marrying family members.
Absence of a family history in a dominantly inherited disease may indicate a de novo mutation
or nonpaternity.
Deviations from Mendelian inheritance may result from uniparental disomy (patient receives
two copies of a chromosome, or part of a chromosome, containing a mutation from one parent
and no copies from the other parent) or genetic imprinting (same abnormal gene inherited from
mother has a different phenotype than that inherited from father as a result of silencing or
imprinting of one parent’s portion of DNA) (see Williams Hematology, 9th ed, Chap. 12).
SEXUAL HISTORY
One should obtain the history of the sexual preferences and practices of the patient.
PHYSICAL EXAMINATION
Special attention should be paid to the following aspects of the physical examination:
Skin: cyanosis, ecchymoses, excoriation, flushing, jaundice, leg ulcers, nail changes, pallor,
petechiae, telangiectases, rashes (eg, lupus erythematosus, leukemia cutis, cutaneous T-cell
lymphoma)
Eyes: jaundice, pallor, plethora, retinal hemorrhages, exudates, or engorgement and
segmentation of retinal veins
Mouth: bleeding, jaundice, mucosal ulceration, pallor, smooth tongue
Lymph nodes: slight enlargement may occur in the inguinal region in healthy adults and in the
cervical region in children. Enlargement elsewhere, or moderate to marked enlargement in
these regions, should be considered abnormal
Chest: sternal and/or rib tenderness
Liver: enlargement
Spleen: enlargement, splenic rub
Joints: swelling, deformities
Neurologic: abnormal mental state, cranial nerve abnormalities, peripheral nerve
abnormalities, spinal cord signs
LABORATORY EVALUATION
The blood should be evaluated, both quantitatively and qualitatively. This is usually achieved
using automated equipment.
Normal blood cell values are presented in Table 1–4. Normal total leukocyte and differential
leukocyte counts are presented in Table 1–5.
Hemoglobin concentration and red cell count are measured directly by automated instruments.
Packed cell volume (hematocrit) is derived from the product of erythrocyte count and the
mean red cell volume. It may also be measured directly by high-speed centrifugation of
anticoagulated blood.
Both the hemoglobin and the hematocrit are based on whole blood and are, therefore,
dependent on plasma volume. If a patient is severely dehydrated, the hemoglobin and
hematocrit will appear higher than if the patient were normovolemic; if the patient is fluid
overloaded, those values will be lower than their actual level when normovolemic.
Mean (red) cell volume (MCV), mean (red) cell hemoglobin (MCH), and mean (red) cell
hemoglobin concentration (MCHC) are determined directly in automated cell analyzers. They
may also be calculated by using the following formulas:
The units are grams of hemoglobin per deciliter (g/dL) of erythrocytes, or a percentage.
The MCH may decrease or increase as a reflection of decreases or increases in red cell
volume as well as actual increases or decreases in red cell hemoglobin concentration. The
MCHC controls for those changes in red cell size, providing a more reliable measurement of
hemoglobin concentration of red cells.
Red cell distribution width (RDW) is calculated by automatic counters and reflects the
variability in red cell size. The term “width” in RDW is misleading; it is a measure of the
coefficient of variation of the volume of the red cells, and not the diameter. It is expressed as a
percent.
RDW = (Standard deviation of MCV ÷ mean MCV) × 100
— Normal values are 11% to 14% of 1.0.
— The presence of anisocytosis may be inferred from an elevated RDW value.
Reticulocyte index. This variable is derived from the reticulocyte count and gives an estimate
of the marrow response to anemia reflecting the red cell production rate.
— The normal marrow with adequate iron availability can increase red cell production two to
three times acutely and four to six times over a longer period of time.
— The reticulocyte index is used to determine if anemia is more likely the result of decreased
red cell production or accelerated destruction in the circulation (hemolysis).
— By convention, hemolysis should be considered if the reticulocyte index is more than two
times the basal value of 1.0.
— This calculation assumes (1) the red cell life span is ~100 days; (2) a normal reticulocyte
is identifiable in the blood with supravital staining for 1 day; (3) the red cell life span is
finite and the oldest 1% of red cells are removed and replaced each day; and (4) a
reticulocyte count of 1% in an individual with a normal red cell count represents the
normal red cell production rate per day thus, 1 is the basal reticulocyte index.
— The reticulocyte index provides the incidence of new red cells released per day as an
estimate of marrow response to anemia.
Consider a patient with a red cell count of 2 × 1012/L and a reticulocyte count of 15%. The
reticulocyte index is calculated as follows:
— Corrected reticulocyte percent = observed reticulocyte percent × observed red cell
count/normal red cell count. Calculation for patient values in this example = 15 × 2.0/5.0 =
6. This adjustment corrects the percent of reticulocytes for the decreased red cells in an
anemic person. This calculation provides the prevalence of reticulocytes, but we want to
know the incidence of reticulocytes (per day).
— In anemia, under the influence of elevated erythropoietin, reticulocytes do not mature in the
marrow for 3 days and then circulate for 1 day before they degrade their ribosomes and
cannot be identified as such. Reticulocytes are released prematurely and thus may be
identifiable in the circulation for 2 or 3 days and not reflect new red cells delivered that
day, as in the normal state.
— The corrected reticulocyte percent must be adjusted for premature release of reticulocytes.
This is done by dividing the corrected reticulocyte percent by a factor related to the
severity of anemia from 1.5 to 3. In practice, the value 2 is usually used as an
approximation.
— Thus, the corrected reticulocyte percent of 6 ÷ 2 results in a reticulocyte index of three
times the basal value, indicating the anemia is hemolytic.
Enumeration of erythrocytes, leukocytes, and platelets can be performed by manual methods by
using diluting pipettes, a specially designed counting chamber, and a light microscope, but an
electronic method provides much more precise data and is now used nearly universally for
blood cell counts.
Leukocyte differential count can be obtained from stained blood films prepared on glass
slides. Automated techniques may be used for screening purposes, in which case abnormal
cells are called out and examined microscopically by an experienced observer. Normal values
for specific leukocyte types in adults are given in Table 1–5. The identifying features of the
various types of normal leukocytes are shown in Figure 1–1 and are detailed in Williams
Hematology, 9th ed, Chap. 2; Chap. 60; Chap. 67; Chap. 73.
Electronic methods that provide rapid and accurate classification of leukocyte types based
largely on the physical properties of the cells have been developed and are in general use as
described in Williams Hematology, 9th ed, Chap. 2.
Properly stained blood films also provide important information on the morphology of
erythrocytes and platelets as well as leukocytes.
Examination of the blood film may reflect the presence of a number of diseases of the blood.
These are listed in Table 1–6.
FIGURE 1–1 Images from a normal blood film showing major leukocyte types. The red cells are normocytic (normal size) and
normochromic (normal hemoglobin content) with normal shape. The scattered platelets are normal in frequency and morphology.
Images are taken from the optimal portion of the blood film for morphologic analysis. A. A platelet caught sitting in the biconcavity
of the red cell in the preparation of the blood film—a segmented (polymorphonuclear) neutrophil and in the inset, a band neutrophil.
This normal finding should not be mistaken for a red cell inclusion. B. A monocyte. C. A small lymphocyte. D. A large granular
lymphocyte. Note that it is larger than the lymphocyte in C with an increased amount of cytoplasm containing scattered eosinophilic
granules. E. An eosinophil. Virtually all normal blood eosinophils are bilobed and filled with relatively large (compared to the
neutrophil) eosinophilic granules. F. Basophil and in inset a basophil that was less degranulated during film preparation, showing
relatively large basophilic granules. The eosinophilic and basophilic granules are readily resolvable by light microscopy (×1000),
whereas the neutrophilic granules are not resolvable, but in the aggregate impart a faint tan coloration to the neutrophil cytoplasm,
quite distinctly different from the blue-gray cytoplasmic coloring of the monocyte and lymphocyte. (Source: Williams Hematology,
9th ed, Chap. 2, Figure 2–4.)
A NIGHT DRIVE.
A DEATH-BED.
Sir Walter lay in his luxurious bed, where everything was arranged with
the perfection of comfort, warmth, softness, lightness, all that wealth could
procure to smooth the downward path. He was not in pain. Even the
restlessness which is worse than pain, which so often makes the last hours
of life miserable, an agony to the watchers, perhaps less so to the sufferer,
had not come to this old man. He lay quite still, with eyes shining
unnaturally bright from amid the curves and puckers of his heavy old
eyelids, with a half smile on his face, and the air of deliverance from all
care which some dying people have. He was dying not of illness, but
because suddenly the supplies of life had failed, the golden cord had
broken, its strands were dropping asunder. The wheels were soon to stand
still, but for the moment that condition of suspense did not seem to be
painful. There was fever in his eyes which threw a certain glamour over
everything about. He had asked that the candles might be lighted, that the
room should be made bright, and had called his daughter to his side.
Perhaps it was only her own anxiety which had made her suppose that he
had asked for Rochford and the papers. At all events, if he had done so, he
did so no more. He held her hand, or rather she held his as she stood by
him, and he lightly patted it with the other of his large, soft, feeble hands.
“You are looking beautiful to-night—as I used to see you—not as you
have been of late. Alicia, you are looking like a queen to-night.”
“Oh, father, dear father, my beauty is all in your eyes.”
“Perhaps, more or less,” he said; “I have fever in my eyes, and that gives
a glory. The lights are all like stars, and my child’s eyes more than all. You
were a beautiful girl, Alicia. I was very proud of you. Nobody but your
father ever knew how sweet you were. You were a little proud outside,
perhaps a little proud. And then we had so much trouble—together, you and
I—”
She said nothing. She had not attained even now to the contemplative
calm which could look back upon that trouble mildly. It brought hard heart-
beats, convulsive throbs of pain to her bosom still. She had silenced him
often by some cry of unsoftened anguish when he had begun so to speak.
But as he lay waiting there, as it were in the vestibule of death, saying his
last words, she could silence him no more.
“Something has occurred to-night,” he said, “that has brought it all back.
What was it, Alicia? Perhaps your ball; the dancing—we’ve not danced
here for long enough—or the music. Music is a thing that is full of
associations; it brings things back. Was there anything more? Yes, I think
there must have been something more.”
She stood looking at him with dumb inexpressive eyes. She could not,
would not say what it was besides, not even now at the last moment, at the
supreme moment. All the opposition of her nature was in this. Love and
pride and sorrow and the bitter sense of disappointment and loss, all joined
together. She met his searching glance, though it was pathetic in its inquiry,
with blank unresponsive eyes. And after awhile in his feebleness he gave up
the inquiry.
“We have gone through a great deal together, you and I—ah, that is so—
only sometimes I think there was a great deal of pride in it, my dear. My
two poor boys—poor boys! I might be hard on them sometimes. There was
the disappointment and the humiliation. God would be kinder to them. He’s
the real father, you know. I feel it by myself. Many and many a time in
these long years my heart has yearned over them. Oh, poor boys, poor silly
boys! had they but known, at least in this their day—Alicia! how could you
and I standing outside know what was passing between God and them when
they lay—as I am lying now?’
“Oh, father, father!” she cried, with an anguish in her voice.
“It is you that are standing outside now, Alicia, alone, poor girl; and you
don’t know what’s passing between God and me. A great deal that I never
could have thought of—like friends, like friends! I feel easy about the boys,
not anxious any longer. After all, you know, they belong to God, too,
although they are foolish and weak. Very likely they are doing better—well,
now—”
“Oh, father!” she cried, with a keen pang of pain at what she thought the
wandering of his mind. “You forget, you forget that they are dead.”
“Dead!” he repeated, slowly. “I don’t forget; but do you know what that
means? We never understand anything till we come to it in this life. I’m
coming very close, but I don’t see—yet—except that it’s very different—
very different—not at all what we thought.”
“Father,” she cried, in the tumult of her thoughts: “oh, tell me something
about yourself! Are you happy—do you feel—do you remember—”
Alicia Penton had said the prayers and received the faith of Christians all
her life, and she wanted, if she could, to recall to the dying man those
formulas which seemed fit for his state, to hear him say that he was
supported in that dread passage by the consolations of the Gospel. But her
lips, unapt to speak upon such subjects, seemed closed, and she could not
find a word to say.
“Happy!” he said, with that mild reflectiveness which seemed to have
come with the approaching end. “It is a long, long time since I’ve been
asked that question. If you mean, am I afraid? No, no; I’m not afraid. I’m—
among friends. I feel—quite pleased about it all. It will be all right,
whatever happens. I don’t seem to have anything to do with it. In my life I
have always felt that I had everything to do with it, Alicia; and so have you,
my dear; it’s your fault, too. We were always setting God right. But it’s far
better this way. I’m an old fellow—an old, old fellow—and I wonder if this
is what is called second childhood, Alicia; for I could feel,” he said, with
the touching laugh of weakness, “as if I were being carried away—in some
one’s arms.”
His heavy eyes, that were still bright with fever, closed with a sort of
smiling peacefulness, then opened again with a little start. “But it seemed to
me just now as if there was something to do—what was there to do?—
before I give myself over. I don’t want to be disturbed, but if there is
something to do—Ah, Gerald, my good fellow, you are here, too.”
Russell Penton had come in to say that the men who had been sent for so
hurriedly, they whose coming was so important, a matter almost of life and
death, had arrived. He had entered the room while Sir Walter was speaking,
but the hush of peace about the bed had stopped on his lips the words he
had been about to say. He came forward and took the other hand, which his
father-in-law, scarcely able to raise it, stretched out toward him faintly with
a smile. “I hope you are better, sir,” he said, mechanically, bending over the
soft helpless hand, and under his breath to his wife, “They are come,” he
said.
She gave him a look of helplessness and dismay, with an appeal in it.
What could be done? Could anything be said of mortal business now?
Could they come in with their papers, with their conflict of human interests
and passion, to this sanctuary of fading life? And yet again, could Alicia
Penton make up her mind to be balked, disappointed, triumphed over in the
end?
“Better—is not the word.” Sir Walter spoke very slowly, pausing
constantly between his broken phrases, his voice very low, but still clear. “I
am well—floating away, you know—carried very softly—in some one’s
arms. You will laugh—at an old fellow. But I don’t feel quite clear if I am
an old fellow, or perhaps—a child.” Then came that fluttering laugh of
weakness, full of pathetic pleasure and weeping and well-being. “But,” he
added, with a deeper drawn, more difficult breath, “you come in quickly.
Tell me—before it’s late. There is something on my mind—like a shadow—
something to do.”
Alicia held his hand fast; she did not move, nor look up; her eyes blank,
introspective, without any light in them, making no reply to him, fixed on
her father’s face; but her whole being quivering with a conflict beyond
describing, good and evil, the noble and the small, contending over her, in a
struggle which felt like death.
A similar struggle, but slighter and fainter was in her husband’s mind;
but in him it was not a mortal conflict, only a question which was best. Was
it right to permit the old man to float away, as he said, without executing a
project which seemed so near to his heart? Because it was not one which
pleased Russell Penton, because he would rather that it should fail, he felt
himself the more bound to his wife that it should not fail through him.
“It seems almost wicked to disturb you, sir,” he said, “but I heard that
you wanted Rochford; if so, he is here.”
Alicia caught her husband by the arm, pressing it almost fiercely with
her hand, leaning her trembling weight upon him. “But not to disturb you,
father,” she cried, with a gasp.
“Ah!” said Sir Walter, “I remember. What was it? I don’t seem to see
anything—except those lights like stars shining; and Alicia, Alicia! How
beautiful she is looking—like a girl—to-night.”
Her husband gave her a strange glance. She was gripping his arm as if
for salvation, clutching it, her breath coming quick; her cheeks with two red
spots of anxiety and excitement; her eyes dull, with no expression in the
intensity of their passion, fixed on her father’s face. The white dressing-
gown which she had thrown on when she was called to him was open a
little at the throat, and showed the gleam of the diamonds which she had not
had time to take off. It was not wonderful that in the old man’s eyes, with
love and fever together in them, Alicia, in her unusual white, should seem
for a moment to have gone back to the dazzle and splendor of youth.
Sir Walter resumed after a moment, as though this little outbreak of
tender admiration were an indulgence which he had permitted himself. “My
mind’s getting very hazy, Gerald—all quite pleasant, the right thing, no
trouble in it, but hazy. I remember, and yet I don’t remember. If I had but
the clew—Rochford?—the young one, not the father. He’s gone, like all the
rest, and now the young one—reigns in his stead. Bring him, and perhaps
I’ll remember. You could tell me, you two, but you’re afraid to disturb me.
What does it matter about disturbing me? a moment—and then—Send for
him; perhaps I’ll remember.”
Alicia would scarcely let her husband go. She looked at him with terror
in her eyes. What was she afraid of? When he withdrew his arm from her
she dropped down suddenly on her knees by her father’s bedside with a low
shuddering cry, and hid her face, pressing her cheek upon the old man’s
hand. The excitement had risen too high. She could bear it no longer.
Complicated with all the aching and trouble of the moment, the bursting of
this last tie of nature, the dearest and longest companionship of her life, to
have that other anxiety, the miserable question of the inheritance, the
triumph or sacrifice of her pride, which yet, even amid the solemnity of
death, moved her more than any other question oh earth—was something
intolerable. It was more than she could bear. She sunk down, partly out of
incapacity to support herself, partly that she could not, dared not, meet her
father’s eyes with their vague and wistful question. “You could tell me, you
two.” He had seen it, then, in her face, though she had made efforts so
determined to banish all sign of comprehension, all answer out of her eyes.
And now, if he insisted, how could she refuse to answer him? and if Gerald
perceived that the old man had found the necessary clew through her, what
would he think of her? That she had preferred her own aggrandizement to
her father’s peace, that she had prompted him on the very edge of the grave
to enrich herself. She could not sustain Sir Walter’s look, nor face the
emergency without at least that passive protection of her husband’s
presence, which for the moment was withdrawn. And Alicia trembled for
the moment when the strangers would come into this sacred room; the
lawyer, and Edward Penton behind him, hesitating, not without feeling (she
knew), looking sadly at the death-bed where lay one whom in his early days
he had looked up to with familiar kindness. Nobody in the world, not even
Gerald, could be so near to him in that moment as Edward Penton. She felt
this even while she trembled at the anticipation of his coming. He was
nearer than any one living. He would bring in with him the shadows of
those two helpless ones disappeared so long out of life. She bethought her
in that moment how it had been usual to say “the three boys.” Was her mind
wandering, too? All these thoughts surged up into her brain in a wild
confusion—the old tenderness, the irritation, the bitter jealous grudge at
him who had outlived the others, the natural longing toward one who could
understand.
Sir Walter was unaffected by any of these thoughts; he felt it all natural
—that the grief of his child should overwhelm her, that the sense of parting
and loss should be profounder on her side than on his. After various efforts
he raised his hand, which was so heavy, which would not obey his will, and
laid it tenderly upon her bowed head. “Alicia, my dear, child, don’t let it
overwhelm you. Who can tell even how small the separation is—as long as
it lasts, and it can not last very long. You must not, you must not, my dear,
be sorry for me. I tell you—it is all pleasant—sweet. I am not—not at all—
sorry for myself. God bless you, my dear. He is so close that when I say
‘God bless you’ it is as if, my love. He Himself was putting out His hand.”
“Oh, father! oh, father!” she repeated, and could say no more.
And he lay with his face turned to her, and his hand feebly smoothing,
stroking her bowed head, as if she had been a child. She was a child to him,
his young Alicia, looking so beautiful after her ball, in which he had seen
her—had he not seen her?—admired of everybody, the fairest, the most
stately, with the Penton diamonds glittering at her white throat as they were
now. He had her in his mind’s eye so distinct, as he had seen her—was it an
hour, was it a life-time ago? His breathing began to be disturbed, becoming
more difficult, and his thoughts to grow more confused. He talked on, in
broken gasps of utterance, more difficult, always more difficult. The fog in
his throat—he began to feel it now; but always in flashes saw the lights
gleaming, and Alicia in full beauty, with her eyes like the stars, and those
other stars, less precious, yet full of luster at her throat. He took no note of
outward things, being more and more absorbed—yet with a dullness which
softened everything, even the difficulty of the breath—in his own
sensations, and in the sweep of the hurrying movement that seemed to be
carrying him away, away, into halcyon seas beyond, into repose and smiling
peace. But the woman kneeling under his hand was as much alive to every
sound and incident as he was dull to them. Nothing muffled her keen sense,
or stilled the flood of thoughts that were pouring through her mind. She
heard, her heart leaping to the sound, steps approaching softly, on tiptoe,
every noise restrained. She heard a low murmur of voices, then the opening
of the door; but she was afraid to lift her head, to startle her father. She
dared not look up to see who was there, or how he took the entrance of the
new-comers. As for Sir Walter, he was almost beyond disturbance. His hand
moved heavily from time to time over her head; sometimes there was a faint
tremble when a breath came harder, nothing more. Would he die so? she
asked herself, making no sign; was it all sealed up forever, the source of life
that had made the light or the darkness of so many other lives. Her own
wildly beating heart seemed to stand still, to stop in the tremendous
suspense.
“Can you hear me?” said her husband’s voice, low and full of emotion.
“Rochford is here, sir; do you want him?”
He shook his head as he spoke to the two awe-stricken men behind.
“Eh!” Sir Walter gave a start as if half awakened. “Who did you say?—I
think—I must have been asleep. Some one who wants me? They’ll excuse a
—a sick old man. Some one—who?—Gerald—whom did you say?”
“Rochford, sir, whom you wanted to see.”
“Rochford! What should I want with Rochford? He’s the—lawyer—the
lawyer. We have had plenty to do with lawyers in our day. Yes—I think
there was something if I could remember. Alicia, where is Alicia?”
She rose up quickly, all those wild sensations in her stilled by this
supreme call. “I am here, father,” she said. Her countenance was perfectly
colorless, except for two spots of red, of excitement and misery, on her
cheeks. Her lips were parched, it was with difficulty she spoke.
“Yes, my love; stand by me till the last. What was it? I feel stronger. I
can attend—to business. Tell me, my child, what it was.”
She stood for a moment speechless, turning her face toward them all
with a look which was awful in its internal struggle. How was she to say it?
How not to say it? Her fate, and the fate of the others, seemed to lie in her
hands. It was not too late. His strength fluctuated from moment to moment,
yet he could do what was needed still.
“Father,” she began, moistening her dry lips, trying to get the words out
of her parched throat.
Sir Walter had opened his heavy eyes. He looked round with a
bewildered, half-smiling look. Suddenly he caught sight of Edward Penton,
who stood lingering, hesitating, half in sympathy, half in resistance, behind.
The dying man gave a little cry of pleasure. “Ah! I remember,” he said.