Life-threatening thrombotic thrombocytopenic purpura (TTP) in a patient with sickle cell-hemoglobin C disease

Abstract

We report a patient with hemoglobin sickle cell-hemoglobin C disease who developed the clinical syndrome of thrombotic thrombocytopenic purpura (TTP) during admission for typical acute pain crisis. The potential for multiorgan involvement secondary to vaso-occlusive crisis complicated the diagnosis and overlapped with the patient's clinical presentation of chronic bone pain and hemolytic anemia. Clinical improvement and normalization of laboratory parameters followed rapidly in response to plasma exchange therapy.

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

“…Likewise Lee et al. reported a grand mal seizure occurring after infusion of 2 units of pRBCs; however, this patient's mental function was already compromised. Neurologic deterioration after RBC infusion was reported in a HgbS/thalassemia heterozygote as well .…”

“…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.…”

Sickle cell disease in the ICU

“…Although no haemodynamic impairment was reported in its original description [18], the occurrence of shock is not uncommon and probably accounts for most of the deaths during SMOF [15 & ,16,17].The absence of haemodynamic impairment might explain the discrepancy between the low mortality rate reported in the original case series [18] and the fact that SMOF appears to be a major cause of death [14,15 & ,16]. A large number of clinical, biological, and autopsy findings highlight many overlaps between SMOF and other non-SCD conditions such as fat embolism and thrombotic microangiopathy syndromes [35][36][37][38][39][40][41][42], especially in patients who worsen despite aggressive and effective transfusion [14, 36,37]. Thus, SMOF better meets the definition of a syndrome that we name 'catastrophic sickle cell syndrome'.…”