Autoimmune hemolytic anemia differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S; Shyam Patel [2], Irfan Dotani [3]

Overview

A variety of conditions comprise the differential diagnosis of autoimmune hemolytic anemia. These include microangiopathic hemolytic anemia, paroxysmal cold hemoglobinuria, paroxysmal nocturnal hemoglobinuria, hereditary spherocytosis, pernicious anemia, and chronic lymphocytic leukemia. The diagnosis of autoimmune hemolytic anemia can sometimes be made by first ruling out these other causes. It is important to distinguish amongst these conditions since the prognosis and treatment of each condition is different.

Differentiating Autoimmune hemolytic anemia from other Diseases

Characteristic	Causes	Pathophysiology	Laboratory abnormalities	Physical examination	Therapy	Other associations
Autoimmune hemolytic anemia	Abnormal immune activation Failure of self vs. non-self recognition Infections Chronic lymphocytic leukemia Non-Hodgkin's lymphoma Rheumatologic disease	Polyclonal antibody production that binds to and targets red blood cells for destruction intravascurly or extravascularly	Positive Coombs' test for IgG Indirect hyperbilirubinemia Reticulocytosis Low haptoglobin Elevated LDH	Pallor Jaundice Shortness of breath Tachypnea	Removal of offending agent Corticosteroids Cyclophosphamide Cyclosporine A Azathioprine Rituximab Splenectomy	Hemolysis can occur at warm or cold temperatures
Microangiopathic hemolytic anemia	Thrombotic thrombocytopenia purpura Hemolytic uremic syndrome Disseminated intravascular coagulation	Formation of small vessel microthombi	Schistocytes on peripheral blood smear Low hemoglobin Thrombocytopenia Elevated creatinine	Bleeding Thrombosis Pyrexia Altered mental status Neurologic deficits Impaired urine output	Treatment of the underlying cause Plasmapheresis if thrombotic thrombocytopenia purpura is the underlying cause	Can be life-threatening pending the underlying cause TTP required immediate treatment
Paroxysmal cold hemoglobinuria	Production of Donath-Landsteiner antibody, triggered by viral or bacterial infection	Biphasic hemolysin (IgG) that binds red blood cells and low temperatures and triggers complement-mediated intravascular hemolysis at warm temperatures	Positive Donath-Landsteiner antibody Microscopic hematuria Negative Coombs' test for IgG or C3d Negative cold agglutinin titer Indirect hyperbilirubinemia Reticulocytosis Low haptoglobin Elevated LDH	Hematuria in the presence of cold weather Jaundice	Removal of offending agent Steroids Alternative immunosuppression	Associated with syphilis Maternal IgG can cross the placenta and affect the fetus
Paroxysmal nocturnal hemoglobinuria	Genetic defect in anchoring proteins for complement factors on red blood cells	Hemolysis due to loss of complement inhibition on the red blood cell surface, which in turn is due to defect in CD55 (decay accelerating factor) and CD59 (membrane inhibitor of reactive lysis) or other glycophosphatidylinositol-anchored proteins on the red blood cell membrane	Absence of CD55 and CD59 by flow cytometry	Splenomegaly Abdominal tenderness Pallor	Eculizumab Immunosuppressive therapy	Associated with myelodysplastic syndrome Associated with mesenteric and portal venous thrombosis Risk for progression to acute myeloid leukemia
Hereditary spherocytosis	Mutation in ankyrin Mutation in alpha- or beta-spectrin Mutation in band 3 Mutation in protein 4.2	Diminished membrane integrity Increased red blood cell fragility Splenic destruction of deformed red blood cells	Positive eosin-5-maleimide binding to red blood cells Positive osmotic fragility testing Spherocytes on peripheral blood smear	Pallor Jaundice Splenomegaly	Removal of offending medication High-dose vitamin B6 (up to 200mg daily) Avoidance of splenectomy Symptomatic transfusion support with iron chelation as needed	Can be autosomal dominant or recessive
Pernicious anemia	Autoimmune gastritis Production of anti-intrinsic factor antibodies Production of anti-parietal cell antibodies	Impaired vitamin B12 absorption due to absence of intrinsic factor	Low vitamin B12 level Presence of anti-intrinsic factor antibodies Presence of anti-parietal cell antibodies	Gastrointestinal discomfort Weakness Tingling Paresthesias	Lifelong vitamin B12 therapy (1000mcg daily)	Associated with diabetes, thyroid disease, vitiligo and other autoimmune conditions
Chronic lymphocytic leukemia	Mutations in hematopoietic stem cells and B lymphocytes	Clonal proliferation of malignant B lymphocytes	Elevated absolute lymphocyte count Anemia (Rai stage III) and thrombocytopenia (Rai stage IV)	Lymph node enlargement Splenomegaly in Rai stage II Hepatomegaly in Rai stage II Pallor Bleeding	Chemotherapy with rituximab Ibrutinib Venetoclax	Secondary autoimmune hemolytic anemia occurs in 10-25% of patients with CLL Treatment with corticosteroids or anti-leukemic therapy will correct the underlying anemia

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