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2016 ; 6
(2
): 89-92
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When the picture is fragmented: Vitamin B12 deficiency masquerading as thrombotic
thrombocytopenic purpura
#MMPMID27308258
Panchabhai TS
; Patil PD
; Riley EC
; Mitchell CK
Int J Crit Illn Inj Sci
2016[Apr]; 6
(2
): 89-92
PMID27308258
show ga
Thrombotic thrombocytopenic purpura (TTP) has high mortality and necessitates
prompt recognition of microangiopathic hemolytic anemia (MAHA) and initiation of
plasmapheresis. We present a challenging diagnostic workup and management of a
42-year-old man who presented with anemia, thrombocytopenia, and schistocytes on
peripheral smear, all pointing to MAHA. Plasmapheresis and steroid therapy were
promptly initiated, but hemolysis continued. Further workup showed megaloblastic
anemia, severe Vitamin B12 deficiency, high iron saturation, and absent
reticulocytosis, none of which could be explained by TTP. Severe Vitamin B12
deficiency can lead to hemolytic anemia from the destruction of red cells in the
marrow that have failed the process of maturation. However, this should not cause
thrombotic microangiopathy. Previous reports of B12 deficiency presenting with
MAHA and a TTP-like manifestation have identified acute hyperhomocysteinemia as a
missing link between B12 deficiency and MAHA, so this possibility was further
explored. Our patient similarly had significantly elevated serum homocysteine
levels, confirming this suspicion of Vitamin B12 deficiency. Vitamin B12
replacement led to normalization of the elevated levels of homocysteine, the
disappearance of schistocytes on the peripheral smear, and resolution of the
microangiopathic hemolysis, thereby confirming the diagnosis. It is pertinent
that intensivists not only know the importance of early recognition and treatment
of TTP but are also familiar with rare conditions that can present in a similar
fashion.