[Acquired amegacaryocytic thrombocytopenic purpura hiding acute myeloid leukemia]. / Un purpura thrombopénique amégacaryocytaire acquis qui cache une leucémie aigue myéloblastique.

Acquired amegakaryocytic thrombocytopenic purpura is a very rare condition characterized by severe thrombocytopenia linked to the reduction or disappearance of megakaryocytes in the bone marrow. It may be primary idiopathic or secondary to many pathological conditions including hematologic disorders. We report the case of a 24-year-old patient admitted for haemorrhagic syndrome caused by immunological thrombocytopenic purpura. The diagnosis was acquired amegakaryocytosis after the failure of corticotherapy and the performance of myelography. The patient was treated with ciclosporin with rapid progression to acute myeloblastic leukemia. The progression of acquired amegakaryocytosis to acute leukemia is reported but it is generally not so rapid and above all it is preceded by myelodysplastic syndrome or medullary aplasia. This study highlights the importance of a close follow-up of these pathologies with a benign-like appearance.

[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.

Diffuse large B-cell lymphoma presenting as large anterior chest wall mass involving pleura and lung: A possible result of post-traumatic chronic inflammation.

A 67 year-old-man was hospitalized due to chronic pain and a large mass on the anterior chest wall. His medical history showed chest trauma in 1970, the reconstitution of the scenario revealed there was blunt trauma with swelling and rib fracture on the same side. Physical examination revealed an isolated large anterior chest wall mass. Chest radiography showed two bilateral irregular masses, chest computed tomography showed a large right chest wall tumor with pleural effusion, nodules of the right upper lobe and tumor of the left lower lobe without mediastinal lymphadenopathy. Whole body exploration showed only the chest disease. Transthoracic biopsy showed inflammatory reaction. Surgical biopsy by anterior thoracotomy of the right mass was performed under general anesthesia. Histological and immunohistological analysis revealed lymphoid diffuse large cell proliferation with positive staining of CD 20, BCL-6 and MUM1, confirming the diagnosis of diffuse large B-cell malignant lymphoma. Chemotherapy based on CHOP-21 (cyclophosphamide, doxorubicin, vincristine, and prednisolone) was administered with good response after three cycles. The patient was discharged under surveillance in good condition after the end of chemotherapy. We report an infrequent neoplasm with an unusual and subtle clinical presentation.

[Gastrointestinal bleeding revealing polycythemia. A case report]. / Hémorragie digestive révélatrice d'une polyglobulie. A propos d'une observation.

PURPOSE: Gastrointestinal bleeding, an uncommon complication of polycythemia can be the inaugural sign in exceptional cases. CASE REPORT: A 35-year-old patient was hospitalized for upper gastrointestinal bleeding. Physical examination and laboratory tests led to the diagnosis of polycythemia (Vaquez disease). Gastroscopy showed an hemorrhagic bulber ulcer. Outcome was favorable after chemotherapy and antinuclear treatment. CONCLUSION: Based on this observation and data in the literature we propose that this unusual presentation would suggest a relationship between polycythemia and ulcer disease.