Classification of hemolytic anemia

Mechanisms of different types of hemolysis

Classification Of Disorders Due To Hemoglobin Abnormalities

I.Hereditary

A.Abnormal hemoglobins (hemoglobinopathies)

1.Sickling and related disorders

2.Unstable hemoglobins

3.Hemoglobins with abnormal oxygen affinities

4.Methemoglobins (hemoglobins M)

B.Thalassemias

1.Thalassemias

2.Thalassemias, including hereditary persistence of fetal hemoglobin

C.Methemoglobinemia due to NADH diaphorase (methemoglobin reductase)

deficiency

II.Acquired

1.Toxic sulfhemoglobinemia

2.Toxic methemoglobinemi

3.Toxic carboxyhemoglobinemia

Hemolysis Due To Antibodies

WARM-ANTIBODY IMMUNOHEMOLYTIC ANEMIA

1.Idiopathic

2.Lymphomas: Chronic lymphocytic leukemia, non-

Hodgkin's lymphomas, Hodgkin's disease (infrequent)

3.SLE and other collagen-vascular diseases

4.Drugs

a.-Methyldopa type (autoantibody to Rh antigens)

b.Penicillin type (stable hapten)

c.Quinidine type (unstable hapten)

5.Postviral infections

6.Other tumors (rare)

COLD-ANTIBODY IMMUNOHEMOLYTIC ANEMIA

1.Cold agglutinin disease

a.Acute: Mycoplasma infection, infectious mononucleosis

b.Chronic: Idiopathic, lymphoma

2.Paroxysmal cold hemoglobinuria

Drugs Causing Hemolysis In Subjects

Deficient In G6PD

Antimalarials: Primaquine, pamaquine, dapsone Sulfonamides: Sulfamethoxazole Nitrofurantoin

Analgesics: Acetanilid

Miscellaneous: Vitamin K (water-soluble form), doxorubicin, methylene blue, nalidixic acid, furazolidone, niridazole, phenazopyridine

Clinical presentation

•Circulatory hypoxic syndrome

•Jaundice

•Tachycardia, dyspnea

•Splenomegaly, hepatomegaly in extravascular hemolysis

•Fever, lumbar pain, dark urine in intravascular

•Gall stones

•Skeletal deformations in children

•Trophic lesions of the extremities

Diagnostics

•Low Hb and RBC

•Reticulocytosis

•High indirect bilirubin

•High LDH

•Low haptoglobin

Principles of treatment

Treatment depends on the cause

Hereditary extravascularSpleenectomy

Allogenous transplantation of hemopoietic cells (Hb-pathies, enzymopathies)Replacement therapy (blood transfusion)

AutoimmineCorticosteroidsIV immunoglobulinsCytostatics

Hemosiderosis prophylaxis:

Avoid frequent blood transfusionsAvoid iron supplementsTreatment with iron chelators

Aplastic anemia

Aplastic anemia is a syndrome of bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia

Clinical presentation of aplastic anemia

•Anemia:

–pallor, headaches, palpitatiions, dyspnea, fatigue, edema

•Hemorrhagic syndrome due to thrombocytopenia

–Gyngival bleeds

–Petechial rash

•Infections due to neutropenia:

–Fever

–Overt infections

–Recurrent infections

–mouth and pharyngeal ulcerations