HEMA

ERYTHROCYTE
ABNORMALITIES
DR. LUCI DIANNE EJERCITO-ELEMEOS, RMT, FPOGS
HOMEOSTASIS
• Under normal circumstances, red cell production and circulating RCM (red cell mass) remain at a
constant level regulated by the erythropoietic mechanism, which functions to maintain the body’s
oxygen requirement.
• If the RCM is excessively either decreased or increased, significant clinical problems occur.
ANEMIA
• Is the term used to denote conditions associated with decrease red cells
• Best defined in reference to a decreased hemoglobin level, as the physiologic consequences and
symptoms are the direct result of the decreased oxygen-carrying capacity of the blood.
ERYTHROCYTOSIS/POLYCYTHEMIA
• Designate conditions involving the presence of too many red cells in the circulation.
• Best defined in relation to hematocrit level above the established reference range.
ABSOLUTE ANEMIA OR RELATIVE ANEMIA
• Absolute anemia--There is true decrease in the RCM.

• Relative anemia—this is secondary to the change in the plasma volume, such that there is a shift of the
extravascular volume to the intravascular compartment, expanding the plasma volume and diluting the
RCM(eg. Pregnancy, hyperproteinemia)
ABSOLUTE/RELATIVE ERYTHROCYTOSIS
• Absolute erythrocytosis- there is a true increase in the RCM

• Relative erythrocytosis- there is decrease in the plasma volume, the RCM is normal (eg.dehydration)
ANEMIA
FOUR CATEGORIES:
A. HYPOPROLIFERATIVE
B. MATURATION DISORDERS
C. HEMOLYTIC DISORDERS
D. BLOOD LOSS
ANEMIA
• CLASSIFICATION:
1. etiologic
2. morphologic
3. physiologic
ANEMIA OF BONE MARROW FAILURE
• APLASTIC ANEMIA
• FANCONI’S ANEMIA
• ACQUIRED PURE RED CELL Aplasia
• DIAMOND-BLACKFAN ANEMIA
• MYELOPHTHISIC ANEMIA
ANEMIA OF SYSTEMIC DISORDERS
• ANEMIA OF CHRONIC RENAL DISEASE

• ANEMIA OF ENDOCRINE DISORDERS
• ANEMIA OF PREGNANCY
APLASTIC ANEMIA
• A condition in which there is a peripheral blood pancytopenia, which is decreased in all cellular
constituents: leukocytes, erythrocytes, and platelets.
• The bone marrow is severely hypoplastic or aplastic.
• There are no immature myeloid cells in the peripheral blood.
• There is absence of splenomegaly.
DIAGNOSTIC CRITERIA FOR SEVERE APLASTIC ANEMIA
• Bone marrow cellularity= < 25% of normal or <50% of normal cellularity with <30% hematopoietic cells
PLUS
Any two of the following:
Peripheral blood:
Granulocytes <0.5 x 109/L
Platelets<20 x 109/L
Anemia with<1% reticulocytes
APLASTIC ANEMIA
• Primary Aplastic Anemia

a. Congenital or Hereditary Fanconi’s anemia
b. Idiopathic Aplastic Anemia
• Secondary Aplastic Anemia
FANCONI’S ANEMIA
• A rare, inherited form of aplastic anemia, first seen in 3 brothers by Fanconi

• Autosomal recessive inheritance characterized as pancytopenia with hypoplastic to aplastic bone
marrow
• Clinical presentation: microcephaly, brown skin pigmentation, short stature, malformations of the
thumbs, malformations of the kidney, genital hypoplasia, internal strabismus and mental retardation
FANCONI’S ANEMIA
• HEMATOLOGIC PICTURE: manifests 5-10 years after birth

-anemia, neutropenia and thrombocytopenia
-anisocytosis and poikilocytosis are seen
- marked increase in Hgb F, with resultant decrease in Hgb A
-osmotic fragility and ESR are increased
Bone marrow- hypoplastic which eventually becomes aplastic
FANCON’S ANEMIA
• Treatment: androgens to stimulate erythropoiesis and corticosteroids

: bone marrow transplantation
PURE RED CELL APLASIA
• Inherited
• Acquired
• Primary
• Secondary
INHERITED PURE RED CELL APLASIA
• Diamond Black-fan anemia (congenital hypoplastic anemia or PRCA)- a rare congenital disorder
diagnosed during infancy or early childhood.
-normocytic, normochromic anemia with normal leukocyte and platelet counts and a marked decrease
in marrow erythroblasts
- etiology
ACQUIRED PURE RED CELL APLASIA
• Primary
Idiopathic
Immune mechanism
Immunoglobulin Inhibitor to RBC precursors
Erythropoeitin inhibitor
ACQUIRED PURE RED CELL APLASIA
• Secondary
Benign Thymomas
Drugs
chemicals
Infections
Hemolytic anemia-aplastic crises
CONGENITAL PURE RED CELL APLASIA
• Diamond-Blackfan anemia
DIAMOND-BLACKFAN ANEMIA
• A rare congenital disorder first described in 1938

• A. normochromic, normocytic anemia, normal leukocyte count and platelet count,
Marked decrease in marrow erythroblasts
• Diagnosed during infancy or early childhood
• Etiology: unknown
DIAMOND –BLACKFAN ANEMIA
• Pallor may be evident at birth and is almost always evident by 1

year of age
• Retarded growth, bone age retardation and failure of secondary
sexual maturation, some have osteoporosis
• Characteristically, they do not demonstrate renal abnormalities
• Laboratory findings: Hb values as low as 1.7 g/dL to 9.4 g/dL

normocytic, normochromic with normal red cell morphology, there
is poor response to erythropoietin, elevated Hgb F
PRINCIPAL CLINICAL AND LABORATORY FACTORS
FEATURES FANCONI DIAMOND-BLACKFAN ANEMIA
Hematologic classification Aplastic Anemia Pure Red Cell Aplasia

Brown skin pigmentation common uncommon
Thumb abnormalities common uncommon
Onset of hematologic 5-10 years <1 year
abnormalities
Bone marrow biopsy Hypoplastic to aplastic Cellular
Bone marrow aspirate Pancytopenia Marked decrease in RBC, normal
WBC and platelets
Cytogenetics Multiple chromosomal No associated abnormalities
abnormalities in many tissues
Management/treatment:
- red cell transfusions are the mainstays of therapy every 3-6 weeks to prevent
the symptoms of anemia
- after months to years of treatment, patients may undergo remissions which
may or may not be maintained
- 40-50% responds to steroid therapy
-bone marrow transplantation is also a potential curative therapy
MYELOPHTHISIC ANEMIA
• A common finding among 94% of cancer patients

• Classified as hypoproliferative anemia, also under anemia of chronic
inflammation or chronic disease.
• In some PATIENTS, the degree of anemia correlates with the degree of tumor
load. In others, there is no correlation between the degree of anemia and the
degree of tumor load.
• Results when the bone marrow is invaded by abnormal cells or replacement

by metastatic carcinoma, however there are cases where the invasion is not
due to metastatic cancer cells but by other cells such as those with miliary
tuberculosis and granulomas.
• Not synonymous with leucoerythroblastic reaction.
• Leukoerythroblastic reaction-is the presence of circulating nucleated RBC and

immature leukocytes in the peripheral blood.
• Diagnosis – thru bone marrow biopsy
• ~Treatment and prognosis will depend on the underlying cause of the anemia.
ANEMIA OF CHRONIC RENAL DISEASE
• A hypoproliferative anemia that can be severe and almost invariably occurs in

patients with chronic renal failure; and have elevated BUN levels
• The principal cause is the inadequate marrow response to anemia because of
decrease production of erythropoietin; some studies have evidence that the
bone marrow may not respond to any form of erythropoietin
• Patients have signs and symptoms of anemia, depending on its severity.

• Uremia or accumulation of waste products in the body is associated with
shortened red cell survival and resultant mild hemolytic anemia
• Normocytic, normochromic anemia (with normal indices)
• Because of the resultant kidney disease, plasma volume is increased,
resulting in an artificially decreased hematocrit.
• Although erythrocyte morphology is usually normal, the uremic condition
occasionally produces abnormal cells such as the BURR CELLS and some
HELMET CELLS
• There is a rough correlation between the degree of renal insufficiency (as

measured by the serum creatinine) and the hematocrit.
• The higher the serum creatinine, the lower the hematocrit and the more
severe the anemia.
• The degree of shortening of the red cell survival is directly proportional to the
creatinine level
• Dialysis therapy
• Successful kidney transplant
• Recombinant human erythropoeitin
ANEMIA OF ENDOCRINE DISORDERS
• Anemia may develop secondary to interference with the normal action of

hormones on red cell production
• Red cells are normochromic, normocytic with the marrow showing only
decreased erythroid production with normal myeloid and megakaryocyte
production
• Hypothyroidism
• Hypoadrenalism (Addison’s Disease)
• Hypogonadism (testosterone-stimulates renal erythropoietin production)
ANEMIA OF PREGNANCY
• There is expansion of the total blood volume which may be a physiologic

protective mechanism against the effects of excessive blood loss at the time
of delivery.
ANEMIA OF MATURATION DISORDERS
• A. MACROCYTIC DISORDERS
1. Pernicious anemia
2. Folate Deficiency anemia
3. Tropical Sprue/Non-Tropical Sprue
4. Congenital Dyserythropoietic anemia
ANEMIA OF MATURATION DISORDERS
• B. MICROCYTIC ANEMIA
• 1. Iron Deficiency anemia
• 2. Anemia of Chronic Infectious, Non-Infectious and Malignant Diseases
• 3.Sideroblastic anemia
• 4.Hereditary Hemochromatosis
ANEMIA OF ABNORMAL GLOBIN DEVELOPMENT
• Alpha Thalassemia
• Beta Thalassemia
• Hereditary Persistence of Fetal Hemoglobin
• Hb-S Beta Thalassemia
• Sickle Cell anemia
HEREDITARY ANEMIA OF INCREASED DESTRUCTION
• Hereditary Spherocytosis
• Hereditary Elliptocytosis
• Hereditary Pyropoikilocytosis
• Hereditary Stomatocytosis/Hydrocytosis
• Pyruvate Kinase Deficiency Anemia
• Glucose-6-Phosphate Dehydrogenase Deficiency Anemia
• Abetalipoproteinemia
NON-IMMUNE HEMOLYTIC ANEMIA
• MICROANGIOPATHIC HEMOLYTIC ANEMIA
• THROMBOTIC THROMBOCYTOPENIC PURPURA
• HEMOLYTIC UREMIC SYNDROME
• DISSEMINATED INTRAVASCUKLAR COAGULATION
• OTHERS:
• HEMOLYTIC ANEMIAS ASSSOCIATED WITH CARDIOVASCULAR PROSTHESIS, AORTIC STENOSIS
• HEMOLYTIC ANEMIAS ASSOCIATED WITH
• PARASITES-MALARIA, TOXOPLASMOSIS
• BACTERIA- CLOSTRIDIUM, STAPHYLOCOCCUS, STREPTOCOCCUS
• ANIMAL BITES
IMMUNE HEMOLYTIC ANEMIA
• PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

• AUTOIMMUNE HEMOLYTIC ANEMIA-COLD AND WARM TYPES
• PAROXYSMAL COLD HEMOGLOBINURIA
• DRUG-INDUCED HEMOLYTIC ANEMIA
• HEMOLYTIC DISEASE OF THE NEWBORN
• HEMOLYTIC TRANSFUSION REACTIONS

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ERYTHROCYTE

• Absolute anemia--There is true decrease in the RCM.

• Absolute erythrocytosis- there is a true increase in the RCM

• ANEMIA OF CHRONIC RENAL DISEASE

• Primary Aplastic Anemia

• A rare, inherited form of aplastic anemia, first seen in 3 brothers by Fanconi

• HEMATOLOGIC PICTURE: manifests 5-10 years after birth

• Treatment: androgens to stimulate erythropoiesis and corticosteroids

• A rare congenital disorder first described in 1938

• Pallor may be evident at birth and is almost always evident by 1

• Laboratory findings: Hb values as low as 1.7 g/dL to 9.4 g/dL

FEATURES FANCONI DIAMOND-BLACKFAN ANEMIA

Hematologic classification Aplastic Anemia Pure Red Cell Aplasia

• A common finding among 94% of cancer patients

• Results when the bone marrow is invaded by abnormal cells or replacement

• Leukoerythroblastic reaction-is the presence of circulating nucleated RBC and

• A hypoproliferative anemia that can be severe and almost invariably occurs in

• Patients have signs and symptoms of anemia, depending on its severity.

• There is a rough correlation between the degree of renal insufficiency (as

• Anemia may develop secondary to interference with the normal action of

• There is expansion of the total blood volume which may be a physiologic

• PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

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