[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.

[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.

Hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma.

BACKGROUND: Hemophagocytic lymphohistiocytosis in adults is often secundary to an infection or a neoplasm. In this last case, T cell lymphomas are the most frequent causes. Hemophagocytic lymphohistiocytosis secundary to a B cell lymphoma has been rarely reported. CASE PRESENTATION: We describe a case of a hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma treated with conventionnal chemotherapy leading to a complete remission. CONCLUSION: Prompt etiologic diagnosis and treatment of hemophagocytic lymphohistiocytosis leads to satisfactory outcome.

Risk factors and scoring system for predicting bacterial resistance to cefepime as used empirically in haematology wards.

OBJECTIVES: Bacterial resistance is of growing concern in haematology wards. As the inappropriate administration of empirical antibacterial may alter survival, we studied risk factors for resistance to our usual empirical first-line antibacterial therapy, cefepime. METHODS: We retrospectively studied 103 first episodes of bacteraemia recorded in our haematology department over 2.5 years. Risk factors for cefepime-resistance were identified by multivariate logistic regression with backward selection (P < 0.05). A scoring system for predicting cefepime-resistance was built on independent factor, with an internal validation by the bootstrap resampling technique. RESULTS: 38 (37%) episodes were due to Gram-negative bacteria. Fifty (49%) were due to bacteria resistant to cefepime. Cefepime resistance was significantly associated with a decreased survival at day 30 (P < 0.05). Three risk factors were independently associated with cefepime-resistance: acute lymphoblastic leukaemia; ≥18 days since hospital admission; and receipt of any ß-lactam in the last month. Patients with ≥2 of these risk factors had a probability of 86% (CI 95%, 25 to 100%) to carry a cefepime-resistant strain. CONCLUSION: Using our scoring system should reduce the indication of very broad antibacterial regimens in the empirical, first-line treatment of febrile hematology patients in more than 80% of the cases.

A rare pediatric case of cutaneous gamma/delta T-cell lymphoma.

Cutaneous γ/δ T-cell lymphoma (CGD-TCL) is a recent entity described in the newly revised World health organization-European organization for research and treatment of cancer classification of cutaneous lymphomas. Only a few cases have been reported, of which two pediatric cases. A 15 years old child with a 6 months history of polyadenopathy, cutaneous lesions, general edema and deterioration of general condition was hospitalized. Results from laboratory testing, cutaneous histopathology and immunohistochemistry showed a primary CGD-TCL. Staging was completed by a total body computed tomography. Therapy was planified with SMILE protocol. It is a highly aggressive tumor resistant to chemotherapy, immunotherapy, and radiation therapy. The GDTCL is characterized by a worse prognosis with a median survival of 15 months. Early diagnosis is essential and aggressive therapy is necessary.

[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.

[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.

Dramatical improvement of chemoresistant bone lymphoma with rituximab.

Non-Hodgkin's lymphoma of the bone is a very rare disease that accounts for approximately 5% of all extranodal non-Hodgkin's lymphomas and for 7-10% of primary bone tumours. We report the case of a 28-year-old man who, in June 2001, presented with a right humerus showing painful destructive lesions with pathological fractures. Biopsy revealed diffuse, large B-cell non-Hodgkin's lymphoma expressing CD20. The patient received six cycles of conventional chemotherapeutic regimen, including cyclophosphamide, doxorubicin, vincristine and prednisone, and VP-16 (etoposide), ifosfamide and mitoxantrone. His arm pain worsened, and x-rays demonstrated progressive disease. He began a trial of rituximab, 750 mg/week, for 4 weeks. There was improvement in pain after the first infusion. Radiographic studies conducted 3 months after rituximab therapy showed marked improvement in his humerus disease. MRI showed a decrease of tumour volume with residual minor signal abnormalities of the bone marrow. He had no evidence of recurrent lymphoma 24 months later.