[Hodgkin's lymphoma associated with thrombotic microangiopathy: case report and literature review]

We report a 51-year-old woman, who presented thrombotic microangiopathy associated with Hodgkin's lymphoma relapse after three years of complete remission. After treatment with corticotherapy, plasmatherapy, rituximab and chemotherapy, complete remission of thrombotic microangiopathy and Hodgkin's lymphoma was observed. Our literature review notes eight cases of thrombotic microangiopathy associated with Hodgkin's lymphoma

[ myelodysplastic syndromes]

There are a group of clonal hematologic disorder, which combine ineffective hematopoiesis and evolution to acute myeloid leukaemia. We conducted a 7 years retrospective. In order to specify the typology of the myelodysplastic syndromes. We included patients presented it diagnosed on the complete blood count and the myelogram between January 2001 and January 2008. Forty patients have been included [29 men, 11 women, sex-ratio 2.63], median age 62 +/- 16 years [23-85]. Nineteen patients only presented a peripheral unilineage cytopenia, fifteen patients had a bicytopnia and five presented a pancytopenia. By using the French Americano-British classification, the myelodysplastic syndromes were distributed in 27% of refractory anemias, 15% of refractory anemias with sideroblast, 25% of refractory anemias with excess of blast, 8% of refractory anemias with excess of blast in transformation, and 24% of chronic myelomonocytic leukemia. By using the criteria of the NOH, distribution is of 37% of refractory anemias [with or without sideroblast], 17% of refractory cytopenia with multilineage dysplasia [with or without sideroblast], 33% of refractory anemias with excess of blast, 3% of Myelodysplastic syndromes associated to the insulated delection 5q - and 10% of acute leukeamias. The score of Bournemouth has been calculated for every patient. The results join those of the literature. The authors will discuss the interest using of one or the other classification of that affection. They note the difficulties of diagnosis of the it needed, the limits between the various entities remain still remain badly known for most the practitioners. The need for a precise and complete diagnosis is essential in order to better classify the Myelodysplastic syndromes, and consequently to evaluate the prognosis and to make a reasoned therapeutic decision of it

[ myeloproliferative syndromes]

There are chronic malignant haemopathy resulting from the attack of a hematopoietic original cell and resulting in monoclonal proliferation, without blocking of cellular maturation, concerning at least one of the three lineages myeloid [granulous, erythroblastic, thrombocytic]. These syndromes gather four affections: chronic myeloid leukaemia, the polycythemia vera, the essential thrombocytaemia and the primitive myelofibrosis. It is about a retrospective study carried out over 6 years period from 2002 to 2007. The data were collected from the registers of the laboratory. The diagnosis of these various myeloproliferative syndromes is based on the data of the complete blood count, the blood smear, and is supplemented when it is necessary by the study of the myelogram and the cytogenetic study of the myeloid cells as it is the case for the Chronic myeloid leukaemia. This work interested 50 cases of myeloproliferative syndromes, distributed as: 44 cases of chronic myeloid leukemia, four cases of polycythemia vera and two cases of primitive myelofibrosis. The means of age were respectively of 42 +/- 15 years, 56 +/- 14 years and of 43 +/- 5 years. There was a clear male prevalence. It was not noted a case of essential thrombocytaemy. The epidemiologic aspect and the clinical signs and biological parameters in the diagnosis of these various myeloprliferative syndromes were evoked and discussed during this work. There are rare chronic malignant haemopathy. There is few data on epidemiology of these affections; their incidence is estimated in the Western counties at 5 to 10 new cases per million inhabitants, each year, according to the type. The diagnosis rests on the clinical and hematological data. There are rare affections, requiring the multidisciplinary collaboration [clinician and hematologist], for best dealt with diagnostic and therapeutic. Their evolution, often pejorative is revealed by clinical signs and above all by disturbances with the complete blood count different from those noted at the time of the initial presentation

Constitutional deficits in coagulation factors

Hereditary deficit of coagulation factors is rare affection. This is a retrospective study which analyse the coagulation parameters of 25 patients with an hereditary deficit. Deficiency on factor V, VII and VIII were the most frequent. Generally without symptoms, hereditary deficiency of coagulation factors must to be diagnosing with a great prudence for prevention of hemorrhagie riskin surgery

[Management of hemorrhagic syndrome]

Bleeding can occur when the vessels are affected by illness or injury. More rarely, a hemorrhagic syndrome can be the consequence of a hereditary or acquired anomaly of the haemostasis. A big number of hemorrhagic diseases are now identified. The notion of familial hemorrhagic disease, the circumstances of intervening of bleedings, as well as their localization, and the using of some drugs can help diagnosis. The diagnosis depends on the clinical analysis and the realization or exploring tests of haemostasis. This article aims at the recall of the different aetiological aspects a haemorhagic syndrome and to propose a pratical way for its management