Thrombotic thrombocytopenic purpura in a patient with sickle cell crisis

Abstract

The combination of sickle cell disease crisis and thrombotic thrombocytopenic purpura has been described only a few times. Here we present the case of a patient with a hemolytic crisis due to sickle cell disease complicated by thrombotic thrombocytopenic purpura. We also review the cases previously reported and compare and contrast them, highlighting diagnostic challenges.

The rate of hemolysis in sickle cell disease correlates with the quantity of active von Willebrand factor in the plasma

“…This notion is supported by the description of a thrombotic thrombocytopenic purpura-like syndrome in SCD patients. 21, 22 The binding of sickle erythrocytes to endothelial cell-immobilized VWF could also increase hemolysis by slowing erythrocyte transit enough to induce hemoglobin deoxygenation and polymerization, consistent with the finding that sickle erythrocyte adhesion to ULVWF was highest at a fluid shear stress of 0.5 dyne/cm 2 , approximately that of postcapillary venules. 23 Hyperreactive VWF probably accumulates in SCD by a combination of increased secretion, defective clearance, and impaired processing by ADAMTS13.…”

Thrombospondin-1 inhibits ADAMTS13 activity in sickle cell disease

“…As TSP1 levels increase further in VOE, 1,11 and TSP1 and vWF are both proposed to participate in sickle cell adhesion, we can hypothesize that further ul-VWF inhibition in particularly severe crises/VOE also might promote ul-VWF-mediated thrombotic complications. Our steady state patients with undetectable ADAMTS13 did not have evidence of thrombotic thrombocytopenic purpura (TTP) or microangiopathic hemolytic anemia; there are, however, several reports of patients with vaso-occlusive complications who develop hallmark findings of TTP, [12][13][14] and similar to those with autoimmune TTP, appear to respond to plasma exchange. 15 In fact, an unexplained fall in platelet counts in ACS is associated with increased risk of neurological deterioration, a hallmark feature of the TTP pentad.…”

Coagulopathy and functional hyposplenism during an episode of thrombotic thrombocytopenic purpura in a HgbS/β+‐thalassemia patient

“…This case report describes an episode of TTP that presented in a patient with HgbS/b + -thalassemia heterozygosity. As has been reported in similar cases [2][3][4], the TTP episode developed after initial presentation of the painful vaso-occlusive crises typical of sickle cell disease (SCD) and delayed treatment with TPE, a modality that raises survival in this condition to 80% [5,6].…”

“…We present here an acute case of TTP in a heterozygote HgbS/ β + ‐thalassemia patient complicated by DVT and functional hyposplenism. Other case reports have described both patients presenting during a sickle cell crisis with subsequent development of TTP , and, even more diagnostically challenging, episodes of TTP masquerading as a pain crisis . Chehal et al.…”

“…We present here an acute case of TTP in a heterozygote HgbS/b + -thalassemia patient complicated by DVT and functional hyposplenism. Other case reports have described both patients presenting during a sickle cell crisis with subsequent development of TTP [2][3][4], and, even more diagnostically challenging, episodes of TTP masquerading as a pain crisis [9][10][11]. Chehal et al and Geigel et al reported cases of multiorgan failure and subsequent remission upon initiation of plasma exchange therapy in patients with similar genetic background [12,13].…”