[Erythroblastopenia and primary myelofibrosis: a very rare association (a case report)]. / L'érythroblastopénie et la myélofibrose primitive: association très rare (à propos d'un cas).

The combination of erythroblastopenia and primary myelofibrosis is very rare. We here report the unusual case of a 76-year-old Moroccan patient followed up since 2018 for idiopathic erythroblastopenia, initially treated with corticotherapy and then with ciclosporin. Two years later, the patient reported bone pain with splenomegaly. Assessment including myelogram, bone marrow biopsy and molecular biology showed myelofibrosis. Etiological assessment of myelofibrosis was negative confirming its primitive nature. The patient received ruxolitinib with transfusion support. Patient´s outcome was favorable and marked by improvement of general condition, splenomegaly and transfusion rate. The association between erythroblastopenia and myeloproliferative disorder is exceptional and only a few cases have been reported in the literature.

Posterior Reversible Encephalopathy Syndrome Secondary to R-CHOP Chemotherapy Regimen.

Chemotherapy-induced posterior reversible encephalopathy (PRES) syndrome is a rare event. Its recurrence after reusing the incriminated molecules remains unpredictable. We report the case of a 58-year-old female patient being followed for a diffuse large B-cell lymphoma treated with rituximab, cyclophosphamide, hydroxydaunorubicin hydrochloride (doxorubicin hydrochloride), vincristine (Oncovin), and prednisone (R-CHOP) regimen. On the fourth day of the first R-CHOP cycle, the patient suddenly developed a headache, bilateral blurred vision, and drowsiness. The next day (day five), the patient had a spontaneously-resolving generalized tonic-clonic seizure associated with postictal bilateral blindness without any other neurological deficiency. Brain magnetic resonance imaging (MRI) revealed an increased bilateral signal intensity involving the cortex and subcortical white matter of the parietal and occipital lobes on the T2-weighted and the T2-weighted fluid-attenuated inversion recovery (FLAIR), which confirmed the diagnosis of PRES) syndrome. After resolution of symptoms, the continuation of the R-CHOP regimen did not lead to a recurrence of the syndrome.

Bleomycin-Induced Lung Toxicity in Hodgkin's Lymphoma: Risk Factors in the Positron Emission Tomography Era.

Introduction Bleomycin is a major antimitotic agent in the first-line treatment for Hodgkin's lymphoma. The main limitation of its use is its pulmonary toxicity. The objectives of this study are to find out the risk factors for the occurrence of bleomycin-induced lung toxicity in patients with Hodgkin's lymphoma and, on the other hand, to determine if positron emission tomography scan is a reliable means of early detection of this toxicity. Methods This is a retrospective study conducted in the clinical Hematology Department of Mohammed V Military Instruction Hospital, Rabat, Morocco. All patients with Hodgkin's lymphoma and treated with a bleomycin-based chemotherapy were included. The impact of different clinical and biological factors on the risk of bleomycin-induced lung toxicity occurrence was assessed using univariate and multivariate logistic regression. The benefit of positron emission tomography, usually performed as part of the re-assessment of Hodgkin's lymphoma after two and four cycles, has been evaluated in the detection of bleomycin-induced lung toxicity. Results Among 124 patients included in the study, 18 (14.5%) patients experienced bleomycin-induced lung toxicity. On multivariate analysis, smoking (p = 0.038) and the use of the ABVD regimen (doxorubicin, bleomycin, vinblastine, and dacarbazine) compared to the escalated BEACOPPe regimen (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) (p = 0.018) were statistically significant risk factors. After two and four courses of therapy, the positron emission tomography was able to predict the occurrence of bleomycin-induced lung toxicity before the appearance of clinical symptoms only in 36.4 % and 12.5% of patients, respectively. Conclusion Studies to identify risk factors for the development of bleomycin-induced lung toxicity are crucial to reduce toxicity in the treatment of Hodgkin's lymphoma. However, two- and four-cycle positron emission tomography scans cannot be considered as a reliable means of early detection of this toxicity.

Splenic marginal zone lymphoma associated with hepatitis B virus infection, remission after viral treatment, and splenectomy: A case report and review of the literature.

We report the case of a patient diagnosed with a splenic marginal zone lymphoma with a simultaneous finding of hepatitis B virus infection, who responded to antiviral treatment and splenectomy. We highlighted this association described in the literature and its possible causal role, as well as the available therapeutic choices.

[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.

Acute Laryngeal Dyspnea as the First Presentation of Mantle Cell Lymphoma: A Case Report and Review of the Literature.

INTRODUCTION: Acute laryngeal dyspnea is a life-threatening emergency, and the causes in adults are most often laryngeal tumors or inflammatory edema. Lymphoma of the larynx and especially the mantle cell type is extremely rare. Case Presentation. We report a case of a 43-year-old woman with no particular pathological history. She presented with progressive dyspnea which has evolved towards an inspiratory bradypnea that worsened until she ultimately required an emergency tracheotomy. Biopsies revealed mantle cell lymphoma. The patient has been staged IVB MIPI 6, and she was treated by immunochemotherapy followed by ASCT. The therapeutic evaluation shows a complete remission, 18 months after, and the patient was always disease free. CONCLUSION: The laryngeal localization of the mantle cell lymphoma is extremely rare; it may present catastrophically with acute airway obstruction. The diagnosis is mostly histological, hence the interest of deep biopsy. Given its rarity, the therapeutic strategy must be discussed case by case in a multidisciplinary consultation meeting.

Bing-Neel syndrome revealing Waldenström's macroglobulinemia: a case report.

INTRODUCTION: Bing-Neel syndrome is a lympho-plasmocytic infiltration of the central nervous system. The Bing-Neel syndrome is a rare entity, which often occurs during the evolution of a Waldenström's macroglobulinemia but can in some cases be the revealing mode of it. OBSERVATION: We report the case of a 56-year-old patient with tumoral form of Bing-Neel syndrome revealing Waldentrom's macroglobulinemia. CONCLUSION: This observation describes a rare entity whose diagnosis and therapeutic management is complex.

[Extramembranous glomerulonephritis and myelodysplastic syndrome: a rare association]. / Glomérulonéphrite extra-membraneuse et syndrome myélodysplasique: une association rare.

Myelodysplastic syndromes may be associated with autoimmune diseases. Renal involvement is rare but, if occurs, it manifests predominantly as glomerular diseases. Extramembranous glomerulonephritis associated with myelodysplastic syndrome has been reported very rarely. We here report the case of a patient presenting with glomerulonephritis associated with anemia, revealing low-risk myelodysplastic syndrome. In the light of this case, we conducted a review of the literature of previously published cases and discussed the pathogenic link between these two entities.

Prognostic factors predicting overall survival in Moroccan patients with myelodysplastic syndrome.

BACKGROUND: Prognostic scores are an indispensable tool in the management of myelodysplastic syndromes. AIM: Identify prognostic factors influencing overall survival of patients with myelodysplastic syndromes. METHODS: We included all patients with myelodysplastic syndromes treated in the clinical hematology department of the military hospital of Rabat (Morocco). RESULTS: Seventy-four patients were included. The sex ratio [M / F] was 1.8. The median age was 65.5 years. The median follow-up was 45 months. The karyotype prognostic was good in 74%. In univariate analysis, prognostic factors influencing overall survival are: Age (p = 0.024), karyotype (p = 0.033), rate of bone marrow blasts (p = 0.002), transfusion dependence (p = 0.002), number of cytopenias (p = 0.001), hemoglobin (p = 0.009) and neutrophil count (p = 0.003). In multivariate analysis the only prognostic factor that influenced overall survival was the karyotype (p = 0.036). Transfusion dependence (p = 0.072) and the rate of marrow blasts (p = 0.107) was close to significance. The WHO Prognostic Scoring System (WPSS), the International Prognostic Scoring System (IPSS) and the Revised International Prognostic Scoring System (RIPSS) influence overall survival with a statistical significance (p <0.001). CONCLUSION: Our study confirms the prognostic value of the karyotype and validated the WPSS, the IPSS and the RIPSS scores as significant predictors of overall survival in Moroccan patients with myelodysplastic syndromes.

[Prolonged molecular response induced by imatinib in Philadelphia positive acute lymphoblastic leukemia A case report and brief review]. / Rémission moléculaire prolongée induite par l'imatinib dans la leucémie aiguë lymphoblastique avec chromosome de Philadelphie Cas clinique et revue de la littérature.

Philadelphia or BCR-ABL positive acute lymphoblastic leukemia (PH+ ALL) is the most common and severe of adult ALL. The only potentially curator treatment remains allogeneic hematopoietic stem cells transplantation (SCT) in first complete remission. The use of imatinib has revolutionized the treatment of chronic myeloid leukemia. Its incorporation into PH + ALL protocols also improved the prognosis of this disease giving better complete remission rates compared to chemotherapy alone. The treatment of patients not eligible for SCT remains controversial. Prolonged use of high dose tyrosine kinase inhibitors (TKI) (ie: imatinib at 600 or 800 mg/j) as maintenance therapy seems to be a reasonable approach. We present a case of prolonged molecular remission of PH+ ALL under TKI alone as maintenance therapy.

[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.

[Association between multiple myeloma and acute myeloid leukemia secondary to myelodysplastic syndrome]. / Association d'un myélome multiple et d'une leucémie aiguë myéloïde secondaire à un syndrome myélodysplasique.

We report a rare case of association of two distinct hematologic malignancies: refractory cytopenia with multilineage dysplasia associated with del(5q) and symptomatic multiple myeloma associated with del(17p) and del(13q). After 16 months, the patient presented an acute leukemic transformation of the myelodysplasic syndrome.

[A hemophagocytic syndrome revealing a Griscelli syndrome type 2]. / Un syndrome d'activation macrophagique révélant un syndrome de Griscelli de type 2.

Griscelli syndrome type 2 is a rare autosomal recessive disorder, due to a mutation in RAB27A gene. It associates partial albinism, silver hair and immune deficiency. We report the case of a 6 year-old boy who was admitted to the Emergency department with severe sepsis complicated by hemophagocytic syndrome. Many clinical and biological criteria leads to the diagnosis of type 2 Griscelli syndrome: consanguineous family, recurrent infection, absence of psychomotor retardation, oculocutaneous albinism, silver hair, occurrence of hemophagocytic syndrome and especially the pathognomonic appearance on microscopic examination of the hair. The absence of giant organelles inclusion in all granulated cells eliminated Chediak-Higashi syndrome.

[Light-chain escape from plateau phase]. / Échappement des chaînes légères de la phase de plateau.

We report the first case of light-chain escape from plateau phase occurring after 7 years of evolution of multiple myeloma in a patient who had never received new molecules. A very good partial response was obtained.

[Signification of Epstein-Barr virus detection in the cerebrospinal fluid of a patient with human immunodeficiency virus related Burkitt lymphoma]. / Signification de la détection du virus d'Epstein-Barr dans le liquide céphalorachidien d'un patient suivi pour un lymphome de Burkitt lié au virus de l'immunodéficience humaine.

The significance of Epstein-Barr virus detection in the cerebrospinal fluid of patients with Burkitt lymphoma is poorly studied. We report the case of a patient with immunodeficiency associated Burkitt lymphoma in complete remission who presented 5 months after the end of treatment, an isolated optic neuritis. Lumbar puncture found lymphocytic meningitis and the viral load of Epstein-Barr virus was 234,000 copies per milliliter in the cerebrospinal fluid. These symptoms could be explained by Epstein-Barr virus meningoencephalitis but the detection of MYC rearrangements in the cerebrospinal fluid confirms the diagnosis of Burkitt lymphoma cerebral relapse. The detection of the Epstein-Barr virus DNA in the cerebrospinal fluid should be interpreted with caution.