[Chronic paraneoplastic inflammatory demyelinating polyradiculoneuritis secondary to nasal natural killer lymphoma]. / Une polyradiculonévrite inflammatoire démyélinisante chronique paranéoplasique secondaire à un lymphome natural killer nasal.

We here report a single case of chronic paraneoplastic inflammatory demyelinating polyradiculoneuritis secondary to a nasal natural killer (NK) non-Hodgkin T lymphoma.

[A monocentric study on the management of patients with myelodysplastic syndromes in Morocco]. / Prise en charge des syndromes myélodysplasiques au Maroc à propos d´une étude mono-centrique.

We conducted a retrospective descriptive analytical study in the Department of Clinical Haematology at the Mohammed V Military Training Hospital in Rabat over a period of 10 years. This study included 76 patients diagnosed with myelodysplastic syndrome (MDS) between 2008 and 2018. The average number of cases per year was 7.6. Out of 76 patients, 57% were men and 43% were women. The average age of our study population was 65.75 ± 12.55. The average age was 66.88 ± 13.10. No cases of profession exposed to disease was reported. Ninety-seven point three percent of patients had primary myelodysplastic syndrome and only 2 or 2.7% had myelodysplastic syndrome secondary to chemotherapy. The average time between the first visit and the diagnosis of myelodysplastic syndrome was, on average, 33.6 days ± 51, with a median of 19 days. The IPSS prognostic score was: low risk in 37.4% of cases, intermediate risk 1 in 46.6% of cases, intermediate risk in 12% of cases and high risk in 4% of cases. Thus, 84% of patients had low-risk MDS and 16% had high-risk MDS. Regular monitoring of patients showed many complications such as bleeding in 13% of patients, infections in 8% of cases, secondary hemochromatosis as a result of iterative transfusions in 6.6% of patients and transformation to acute myeloid leukemia in 2.7% of patients. In our study, abstention was the therapeutic choice in 42.1% of patients, transfusion was recommended in 35.5% of patients: red cells in 70% of cases, platelet concentrates in 40% of cases, iron chelators in 25% of transfused patients and EPO in 27% of patients. azacitidine was prescribed in 18% of patients, 50% had low-risk MDS and 50% had high-risk MDS. Bone marrow transplant was the only curative treatment for MDS. It was performed in a single patient with high risk MDS.

[Treatment of systemic AL amyloidosis: about 25 cases]. / Traitement des amyloses AL systémiques: à propos de 25 cas.

Primarye systemic AL amyloidosis is a rare hematologic disorder. The majority of the therapeutic guidelines are based on phase II studies or on retrospective comparisons and case series. Our study aimed to describe all the cases of primary AL amyloidosis reported in 2 military hospitals and to make a comparison between standard melphalan-dexamethasone protocol and new agents in first-line treatment of patients with this disease. We conducted a retrospective, descriptive and multicentric study of all patients with AL amyloidosis whose data were collected during the period July 2009-June 2016. Twenty five patients were enrolled in the study (12 patients treated with melphalan-dexamethasone and 13 with bortezomib-based protocol or lenalidomide-based protocol). There was no significant difference in the epidemiological, clinical and prognostic features between the 2 groups. After a median follow up of 40 months, median overall survival was 54 months in the melphalan-dexamethasone-treated group and 60 months in the new therapies-treated group (P = 0.98). Progression-free survival was 18 months in the standard treatment group vs 11 months in the 2nd group (p = 0.08). In our small case series we haven't found a superiority of the new therapies compared to the standard protocol. This result should be confirmed by a true prospective study, mainly because of the cost of these new molecules that are not always accessible, especially in developing countries.

[Plasma cell leukemia: three case-reports and review of literature]. / La leucémie à plasmocytes : à propos de trois cas et revue de la littérature.

Plasma cell leukemia (LP) is a rare hematologic malignancy. Its prognosis is very derogatory. It is defined by the presence in circulating blood of more than 2 G/L plasmocytes or greater than 20% of the total leukocytes. It comes in two forms: secondary plasma cell leukemia complicating multiple myeloma (MM) and primary setting. Its incidence is estimated at 0.9% of patients with acute leukemia and 2-4% of patients with MM. We report, through three observations, the clinical presentation of the plasma cell leukemia, its cytological features, immunophenotypic, physiopathological and therapeutic care.