A Rare Cause of Thrombotic Thrombocytopenia Purpura- (TTP-) Like Syndrome, Vitamin B12 Deficiency: Interpretation of Significant Pathological Findings

Abstract

Thrombotic thrombocytopenia purpura (TTP) is a hematological emergency that requires rapid assessment followed by prompt initiation of therapy due to high mortality associated with delayed treatment. TTP has many causes including heritable syndromes, ADAMTS13 deficiency, and drugs-related etiologies. Profound vitamin B12 deficiency can, in rare cases, mimic TTP in presentation, and since plasmapheresis can be of limited benefit, prompt diagnosis is necessary for accurate treatment with B12. Therefore, careful analysis of all clinical signs, symptoms, and labs must be assessed. We report a patient who presented with a diagnosis of TTP, and repeat assessment confirmed a diagnosis of sever vitamin B12 (B12) deficiency with pancytopenia who was appropriately treated with B12.

Fooled by the fragments: vitamin B12 deficiency masquerading as thrombotic thrombocytopenic purpura

“…Intramedullary hemolysis secondary to pseudo-folate deficiency and subsequently impaired DNA synthesis and cellular division manifests in one of two mechanisms. One mechanism may be that immature reticulocytes arrest in development and are destroyed within the marrow [ 7 , 8 ]. An alternative mechanism is impaired marrow egression.…”

Suspected Metformin-induced Cobalamin Deficiency Mimicking Thrombotic Thrombocytopenic Purpura

“…Cobalamin deficiency cannot only lead to ineffective erythropoiesis but can also increase red blood cell rigidity, which can in turn promote intramedullary destruction of red cell precursors [7]. As the erythrocyte precursor cells are nucleated and contain very little hemoglobin, lysis of these cells translates into laboratory findings of very high LDH (>2,500 IU/L), reticulocytopenia and relatively normal bilirubin which can be used to distinguish from TTP [1,8]. Additionally, cobalamin deficiency can increase homocysteine levels in the blood, which is associated with endothelial injury, vasoconstriction, stimulation of the coagulation cascade and increased platelet aggregation, all of which can promote intravascular hemolysis [7].…”

Pernicious anemia: Pathophysiology and diagnostic difficulties

“…48 In a study of 201 patients with CD, pseudo-TMA was reported in 2.5% of patients, 45,49 which can potentially lead to misdiagnosis of thrombotic thrombocytopenic purpura (TTP) and wrong treatment with plasma exchange. 50,51 One study depicted that pseudo-TMA patients had higher mean LDH levels, higher mean platelet counts, lower mean reticulocyte count, and lower mean neutrophil counts, compared to patients with true TTP. Other clues on PBS examination pointing against the diagnosis of TTP include the presence of HSNs and macroovalocytes.…”

“…Cytoskeletal fragility of erythroblasts may contribute to schistocyte formation in megaloblastic anemia, depending on the severity of dyserythropoiesis 48 . In a study of 201 patients with CD, pseudo‐TMA was reported in 2.5% of patients, 45,49 which can potentially lead to misdiagnosis of thrombotic thrombocytopenic purpura (TTP) and wrong treatment with plasma exchange 50,51 . One study depicted that pseudo‐TMA patients had higher mean LDH levels, higher mean platelet counts, lower mean reticulocyte count, and lower mean neutrophil counts, compared to patients with true TTP.…”