Hemolytic anemia diagnosis

TabbaralA. Hemolytic anemias diagnosis and management. Med Clin North Am 1992 76 649-669. 

Laboratory investigations that aid in the diagnosis of hemolytic anemia are listed in Table 52-7. 

Kleinfeld (1980) reported a case of arsine poisoning in a 31-y-old man. The exposure to arsine occurred from a leaking canister thought to be empty. The exposure duration was estimated to be 1-2 min, but no actual or estimated arsine concentrations were available. The victim presented with hematuria. On hospital admission, no intact erythrocytes were present in the urine, hematocrit was 43%, and hemoglobin was 9.8 g/ dL. The hematocrit dropped to as low as 18%, the correction of which required one unit of packed cells. Based upon the exposure history and the subject s note of a "garlicky" odor, the diagnosis was arsine-induced hemolytic anemia. Urinary arsenic was 7.2 mg/L on admission and 0.1 mg/L 4 d later. The patient was subsequently discharged. 

Barr virus confirmed that he had infectious mononucleosis. He was kept in bed for 2 weeks, by which time the signs of inflammation and hepatocellular damage entirely disappeared. However, mild anemia (hemoglobin 110-120 g/L) with reticulocytosis (50%-6.0%), increased serum unconjugated bilirubin, and splenomegaly still remained, suggesting the presence of persistent hemolysis. The physician therefore performed further examinations to confirm the diagnosis of hemolytic anemia. 

G6PD deficiency is the most common enzymatic disorder of red blood cells and should be considered in the differential diagnosis of any case of hemolytic anemia. It affects 400 million people worldwide with a wide variation in prevalence. It is very rare in the indigenous populations of northern Europe but increases to frequencies of 20% in parts of southern Europe, Africa, and Asia and up to 40% in certain areas of Southeast Asia and the Middle East. 

Thrombotic thrombocytopenic purpura is a rare acute or subacute disease in adults, rather similar to the hemolytic uremic syndrome in children, in which there is systemic malaise, fever, skin purpura, renal failure, hematuria and proteinuria. Hemorrhagic infarcts caused by platelet microthrombi occur in many organs in the brain they may cause stroke-like episodes (Matijevic and Wu 2006) although more commonly there is global encephalopathy. The blood film shows thrombocytopenia, hemolytic anemia and fragmented red cells. The differential diagnosis includes infective endocarditis, idiopathic thrombocytopenia, heparin-induced thrombocytopenia with thrombosis, systemic lupus erythematosus, non-bacterial thrombotic endocarditis and disseminated intravascular coagulation. 

D-7) Pyruvate kinase deficiency. The cell can not produce the ATP that normally is pr uced during this reaction and is needed for the cell s sodium/potassium pump. There is a hemolytic anemia. The diagnosis can be made by assay for pyruvate kinase in red blood cells. The inheritance is autosomal recessive. 

I. LCAT deficiency. Cholesterol associated with HDL cannot be esterified. There is a buildup of unesterified cholesterol, with comeal opacities, renal insufficiency, hemolytic anemia, and premature atherosclerosis. The diagnosis may be made on enzyme assay for plasma LCAT. 

Three patients developed a hypersensitivity syndrome after taking pyrimethamine 12.5 mg + dapsone 100 mg weekly as malaria prophylaxis (16). The diagnosis was based on the presence of fever, lymphadenopathy, a maculopapular rash, and hepatitis. A mild Coombs -positive hemolytic anemia was also observed in one of the patients. All the clinical, hematological, and biochemical abnormalities normalized within 3 months of tapering regimens of moderate-dose prednisolone. 

Hirono A, Forman L, Beutler E. Enzymatic diagnosis in non-spherocytic hemolytic anemia. Medicine (Baltimore) 1988 67 110-7. 

Reference intervals are given in Table 32-3. Total erythrocyte porphyrin concentrations are increased in EPP, CEP, the rare homozygous variants of the autosomal dominant porphyrias, iron deficiency, hemolytic anemia, some other forms of anemia, and lead poisoning. A normal total porphyrin concentration excludes the diagnosis of EPP. Distinction between EPP and other causes of increased erythrocyte total porphyrin concentration requires differentiation between protoporphyrin and its zinc chelate because the acidic condition of this assay dissociates the zinc chelate and provides only a measure of total porphyrin. 

The clinical importance of pyruvate kinase lies in the diagnosis of pyruvate kinase deficiency in erythrocytes, which is the second most common congenital enzyme defect and leads to chronic hemolytic anemia. 

Differential diagnosis of sphero-cytes in the peripheral blood includes warm AIHA (autoimmune hemolytic anemia) and hereditary spherocytosis. Use the osmotic fragility test (HS) and direct Coombs test (AIHA) to tell them apart. 

Chloroalkylamine leukemia alkylating agents immune hemolytic anemia bms Tc immune complex reactions Diagnosis"... 

The authors proposed that for the prompt diagnosis of drug-induced hemolytic anemia, tests for all causative drugs should be conducted by two methods. 

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