TTP was diagnosed in this patient owing to the presence of thrombocytopenia, MAHA with schistocytes
of 3% and neurological manifestations.
Important distinguishing features that may assist in diagnosing TTP in this context include the presence of schistocytes
on peripheral smear, a negative Coombs test, normal complement levels, and a low level of ADAMTS13 activity.
Microangiopathic hemolysis ([greater than or equal to]1% schistocytes
plus helmet cells and the presence of any number of spherocytes)
are defined by the presence of fragmented and/or degenerated RBCs within the LP capillaries.
The peripheral blood smear can show up to 10% schistocytes
, along with helmet cells, which are produced due to damage of the endothelial layer of small vessels, resulting in fibrin deposition and platelet aggregation.
The diagnosis of thrombotic thrombocytopenic purpura was confirmed after exclusion of many of the aetiological factors of postoperative digital ischaemia, a positive haemolytic screen and blood film examination which showed schistocytes
and fragmented red cells.
4 BUN (mg/dL) 28 8-84 LDH (units/L) 1,080 131-3,007 ADAMTS13 activity level (%) 90 * 84-131 Schistocytes
present 8 -- (no.
Blood smear examination demonstrated red blood cell fragmentation, a few schistocytes
The clinical presentation of all of our cases were consistent with TTP (thrombocytopenia, microangiopathic hemolysis with schistocytes
in the peripheral blood smear, elevated LDH) and supported by severe ADAMTS-13 deficiency (< 5%).
Peripheral smear revealed basophilic stippling, nucleated red blood cells, and absence of schistocytes
Blood peripheral smear from the day of admission was significant for the schistocytes
(Figure 1) suggesting ongoing haemolysis.