Abstract
Introduction. Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Case Report. A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests' results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear. Low ADAMTS13 activity (<10%) with elevated ADAMTS13 antibody clinched the diagnosis of severe acquired TTP, and plasmapheresis was started. There was an initial improvement in his hematological markers, which were however not sustained on discontinuation of plasmapheresis. For his refractory TTP, he was resumed on daily plasmapheresis and Rituximab was started. Furthermore, the initial serum Vitamin B12 and reticulocyte index were low in the presence of anti-intrinsic factor antibody. So with the concomitant diagnosis of pernicious anemia, Vitamin B12 was supplemented. The rest of the immunological workups were negative. Subsequently, his symptoms resolved and his hematological parameters improved. Discussion. While pernicious anemia can masquerade as TTP, an actual association between the two can also occur and needs further evaluation and characterization.
Highlights
Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders
Multiple cases of pernicious anemia and the resulting Vitamin B12 deficiency presenting with microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, and masquerading as TTP, have been described [14,15,16,17,18]
ADAMTS13 activity of less than 10% with elevated ADAMTS13 antibody (>140 u/mL, normal < 12 u/mL) clinched the diagnosis of severe acquired TTP, and the patient was started on plasmapheresis
Summary
Thrombotic thrombocytopenic purpura (TTP) is a rare hematological disorder, characterized by microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, leading to end organ damage like fever, renal dysfunction, and neurological manifestations [1]. Its association with pernicious anemia has rarely been reported. Multiple cases of pernicious anemia and the resulting Vitamin B12 deficiency presenting with MAHA and thrombocytopenia, and masquerading as TTP, have been described [14,15,16,17,18]. The actual association of pernicious anemia and TTP, with the two entities being present simultaneously in a patient, has rarely been reported. We describe a case of a young man with severe acquired TTP, who was concurrently diagnosed with pernicious anemia
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