Thrombotic Thrombocytopenic Purpura and Multiorgan System Failure in a Child With Sickle Cell-Hemoglobin C Disease

Majjiga, Venkata Sasidhar; Tripathy, Asit K.; Viswanathan, Kusum; Shukla, Mayank

doi:10.1177/0009922809338314

Cited by 7 publications

(3 citation statements)

References 16 publications

(27 reference statements)

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“…TPE has been previously used successfully to clear circulating cytokines in cases of macrophage activation syndrome or "cytokine storm" syndrome complicating rheumatological conditions, as well as in the context of acute liver failure for the same purpose [22]. There are several case reports and case series on the safe use of TPE in patients with SCD both in the context of multiorgan failure [23], as well as in the context of microangiopathic haemolytic anaemia (MAHA)/thrombotic thrombocytopenic purpura (TTP), with excellent clinical outcomes [24][25][26][27][28]. Interestingly, all cases reported as TTP also had severe respiratory failure, which is not a feature of TTP, all cases received red cell transfusion before TPE, and ADAMTS 13 levels were not tested at all or tested after treatment and found to be normal.…”

Section: Therapeutic Plasma Exchangementioning

confidence: 99%

Fat Embolism Syndrome in Sickle Cell Disease

Tsitsikas

Vize

Abukar

2020

JCM

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Fat embolism syndrome is a devastating complication of sickle cell disease resulting from extensive bone marrow necrosis and associated with high mortality rates, while survivors often suffer severe neurological sequelae. Despite that, the syndrome remains under-recognised and under-diagnosed. Paradoxically, it affects exclusively patients with mild forms of sickle cell disease, predominantly HbSC and HbSβ+. A significant number of cases occur in the context of human parvovirus B19 infection. We provide here a brief summary of the existing literature and describe our experience treating 8 patients in our institution. One patient had HbSS, 6 HbSC and 1 HbSβ+. All patients developed type I respiratory failure and neurological involvement either at presentation or within the first 72 h. The most striking laboratory abnormality was a 100-fold increase of the serum ferritin from baseline. Seven patients received emergency red cell exchange and 1 simple transfusion. Two patients (25%) died, 2 patients (25%) suffered severe neurological impairment and 1 (12%) mild neurological impairment on discharge, while 3 (38%) patients made a complete recovery. With long-term follow-up, 1 patient with severe neurological impairment and one patient with mild neurological impairment made dramatic improvements, making the long-term complete recovery or near complete recovery rate 63%. Immediate red cell exchange transfusion can be lifesaving and should be instituted as soon as the syndrome is suspected. However, as the outcomes remain unsatisfactory despite the increasing use of red cell exchange, we suggest additional therapeutic measures such as therapeutic plasma exchange and pre-emptive transfusion for high risk patients.

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Section: Therapeutic Plasma Exchangementioning

confidence: 99%

Fat Embolism Syndrome in Sickle Cell Disease

Tsitsikas

Vize

Abukar

2020

JCM

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“…This can be achieved by transfusing to keep the HbS level as low as possible (< 10%) although strong evidence to support this stringent threshold is not available. There are multiple reports of patients with ACS or MOFS who develop worrisome findings of thrombotic thrombocytopenic purpura (TTP), [49][50][51][52][53][54] a microangiopathic hemolytic anemia characterized by RBC fragments (schistocytes), thrombocytopenia, and microthrombosis, and classically due to deficiency or inhibition of ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13), a protein responsible for cleavage of thrombogenic high molecular weight von Willebrand factor multimers. The mechanisms driving a TTP-like syndrome in SCD are unknown, although alterations in von Willebrand factor and ADAMTS13 homeostasis have been reported.…”

Section: Acute Chest Syndromementioning

confidence: 99%

Crises in Sickle Cell Disease

Novelli

Gladwin

2016

Chest

107

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In spite of significant strides in the treatment of sickle cell disease (SCD), SCD crises are still responsible for high morbidity and early mortality. While most patients initially seek care in the acute setting for a seemingly uncomplicated pain episode (pain crisis or vaso-occlusive crisis), this initial event is the primary risk factor for potentially life-threatening complications. The pathophysiological basis of these illnesses is end-organ ischemia and infarction combined with the downstream effects of hemolysis that results from red blood cell sickling. These pathological changes can occur acutely and lead to a dramatic clinical presentation, but are frequently superimposed over a milieu of chronic vasculopathy, immune dysregulation, and decreased functional reserve. In the lungs, acute chest syndrome is a particularly ominous lung injury syndrome with a complex pathogenesis and potentially devastating sequelae, but all organ systems can be affected. It is, therefore, critical to understand the SCD patients' susceptibility to acute complications and their risk factors so that they can be recognized promptly and managed effectively. Blood transfusions remain the mainstay of therapy for all severe acute crises. Recommendations and indications for the safest and most efficient implementation of transfusion strategies in the critical care setting are therefore presented and discussed, together with their pitfalls and potential future therapeutic alternatives. In particular, the importance of extended phenotypic red blood cell matching cannot be overemphasized, due to the high prevalence of severe complications from red cell alloimmunization in SCD.

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“…Even though it is not part of standard management for SCD complications, there are previous published case reports and case series where it has been used successfully. TPE was used successfully in a case of ACS with suboptimal response to RCE [13], in a number of cases of presumed thrombotic thrombocytopenic purpura [14][15][16][17][18], while there are two published case series where TPE was used successfully in the management of severe multiorgan failure [19,20]. This is a single Centre experience of a small number of cases.…”

Section: Discussionmentioning

confidence: 99%