Purpura thrombotique thrombocytopénique et autres syndromes de microangiopathie thrombotique au cours de l’infection par le virus de l’immunodéficience humaine

Human immunodeficiency virus (HIV) infection represents a risk factor for thrombotic microangiopathy. HIV-associated thrombotic microangiopathies encompass two entities with distinct pathophysiology, clinical presentation, treatment and prognosis. Thrombotic thrombocytopenic purpura associated with human immunodeficiency virus is typically characterized by a sudden onset in a patient with a moderate immune deficiency and a few events of opportunistic diseases, and a profound acquired deficiency in the von Willebrand factor cleaving protease ADAMTS13. This diagnosis requires a well-codified management including daily therapeutic plasma exchanges, a highly active antiretroviral therapy and eventually immunomodulatory drugs. The prognosis is good with a response rate and an overall survival comparable to that of HIV-negative thrombotic thrombocytopenic purpura. On the opposite, HIV-associated thrombotic microangiopathy with a progressive onset that occurs in profoundly immunocompromised patients with past history of multiple opportunistic diseases usually have a detectable ADAMTS13 activity and a worse prognosis. Usual treatment is poorly efficient. Forthcoming studies should assess the role of immunomodulatory drugs such as rituximab in the setting of HIV-associated thrombotic microangiopathy, and identify possible risk factors associated with the occurrence of these diseases.     

Late vascular complications after cranial radiotherapy: A report of two illustrative cases

Cranial radiotherapy (CRT) is used to treat a large variety of benign and malignant disorders. We present two cases of late neurological complications after CRT and briefly discuss its diagnosis and their shared pathophysiological aspects. The first case is a patient with cognitive impairment associated to mineralizing microangiopathy ten years after CRT for nasopharyngeal carcinoma and the second one is a woman with Stroke-like Migraine Attacks after Radiation Therapy (SMART) syndrome two years after CRT for anaplastic meningioma. Nowadays, higher survival rates might cause an increase in appearance of late neurological complications after CTR. These reported cases show that late complications can mimic a wide variety of neurological conditions and the importance of magnetic resonance image (MRI) to get a diagnosis.     

Un syndrome POEMS révélé par un syndrome des orteils bleus

Introduction

Arterial and venous thromboses occur in almost one in five patients with POEMS syndrome and usually in macrocirculation.

Hypersignaux de la substance blanche révélant un pseudoxanthome élastique

Pseudoxanthoma elasticum (PXE) is an inherited connective tissue disease characterized by skin, eye, cardiovascular, and, less often, cerebrovascular manifestations. We report the case of a 32-year-old woman who presented with fortuitously discovered cerebral white matter lesions. The pattern of the lesions was compatible with vascular leucopathy. Neurological examination, CSF and biological assessment were normal. Physical examination demonstrated cutaneous lesions characterized by yellowish papules in the neck and axilla with calcification of elastic fibres showed on the skin biopsy and retinal angioid streaks, which made PXE a plausible diagnosis. Sequencing of the ABCC6 gene confirmed PXE. Thus, PXE must be considered when confronted with cerebral microangiopathy of undetermined origin.     

Microangiopathies thrombotiques secondaires

Thrombotic microangiopathies (TMA) are termed secondary when associated to a specific context favouring their occurrence. They encompass mainly TMA associated with pregnancy, allogeneic hematopoietic stem cell transplantation, cancer, drugs, or HIV infection. Secondary TMA represent a heterogeneous group of diseases which clinical presentation largely depends on the associated context. It is therefore mandatory to recognize these conditions since they have a significant impact in TMA management and prognosis. A successful management still represents a challenge in secondary TMA. Significant progresses have been made in the understanding of pregnancy-associated TMA, allowing an improvement of prognosis; on the opposite, other forms of secondary TMA such as hematopoietic stem cell transplantation-associated TMA or TMA associated with chemotherapy remain of dismal prognosis. A better understanding of pathophysiology in these forms of TMA, in association with a more empirical approach through the use of new therapeutic agents that can also help in the understanding on new mechanisms a posteriori, should improve their prognosis. The preliminary encouraging results reported with complement blockers in this field could represent a convincing example.     

Microangiopathie thrombotique de novo post-transplantation rénale révélant un déficit en facteur H et en facteur I

A 52-year-old man with an end stage renal failure of undetermined aetiology was hemodialysed in 2002. He received a cadaveric kidney transplantation in 2004. After an episode of diarrhea, a thrombotic microangiopathy was diagnosed in July 2009 and during this episode, a low C3 serum level was identified. Plasma exchanges were beneficial. Exploration of the low C3 serum level revealed both factor H and factor I deficiencies. We think that the renal failure of undetermined aetiology was probably an unnoticed haemolytic and uremic syndrome which recurred more than five years after transplantation.     

Le purpura thrombotique thrombocytopénique à la Martinique : étude rétrospective entre 2008 et 2015

Introduction

Some studies suggest that thrombotic thrombocytopenic purpura (TTP) occurs more often in African Americans. However there is low evidence for this in the literature. The aim of our study was to describe the clinical and biological characteristics of TTP in the Afro-Caribbean population of Martinique.