A 3 1 -year-old graduate studen t From Africa who immigrated to the Unites States 6 mon ths previously presen ts to the emergency departmen t complaining of general-ized fatigue and dark urine For 1 -week duration . Patien t denies Fever or recent infec-tions. Physical exami nation reveals an alert and Ful ly orien ted Female with pal lor.
Laboratory workup shows hemoglobin of LJ.1 g/dL, mean corpuscular volume (MCV) of 1 06 FL. leukocyte count of 7,500/,LLL. platelet count of 3LJ,OOO/,LLL. and LDH of 3,700 U/L. Urine is grossly dark, and analysis reveals 1 -2 RBC/HPF Direct an tiglobin test is negative. Peripheral blood smear is shown below.
What is the most likely diagnosis?
- Hemoglobin H disease
- Beta thalassemia major
- Aplastic anemia (AA)
- Thrombotic thrombocytopenic purpura (TTP)
- Autoimmune hemolytic anemia (AIHA)
D. Peripheral slide reveals increased number of fragmented red blood cells (RBC) schistocytes. This is characteristic of microangiopathic hemolytic anemia (MAHA). The clinical picture ofMAHA, thrombocytopenia, negative direct antiglobin test, and hemoglobinuria (1-2 RBC/ HPF in urine) supports the diagnosis ofTTP.
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