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Fooled by the Fragments: Masquerading Microangiopathy.

PubMed(2010)

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摘要
VOLUME 93 NO. 1 JANUARY 2010 The hematologic aberrations seen in cobalamin deficiency can include pancytopenia, the presence of hypersegmented neutrophils and macroovalocytes on peripheral blood smear, and elevated serum levels of LDH and bilirubin. As seen in the present case, severe cobalamin deficiency can also present with a clinical and hematological picture similar to a microangiopathic hemolytic process. In addition, neuropsychiatric manifestations of cobalamin deficiency are marked by paresthesias, ataxia, urinary and fecal incontinence, impotence, optic atrophy, memory loss, dementia, and various psychiatric disorders including depression, hallucinations, and personality changes. Schistocyte formation in cobalamin deficiency may result from increased membrane rigidity with reduced deformability and subsequent lysis as RBCs pass through the reticuloendothelial system . The anemia of cobalamin deficiency is thought to result from a combination of lack of production and increased destruction of RBCs while thrombocytopenia is caused by a lack of production of platelets. We believe that in cases of apparent TTP with high MCV or other data suggestive of cobalamin deficiency, serum cobalamin levels should always be checked. If a suspicion for cobalamin deficiency arises in the evaluation of TTP, empiric treatment with infusion of FFP can be used until more definitive testing is performed. PEX may be associated with serious side effects. In a nine-year cohort study of 206 consecutive patients treated for TTP, 5 of the 206 (2%) died of complications of PEX treatment. Fifty-three patients (26%) had major complications attributed to PEX treatment, including systemic infection, venous thrombosis, and hypotension requiring blood pressure support . With review of a peFooled By the Fragments: Masquerading Microangiopathy
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