Solid tumors after chronic lymphocytic leukemia patients: Report of six cases and review of the literature.

INTRODUCTION: Malignancies have been reported to occur with increased frequency in chronic lymphocytic Leukemia (CLL) patients. The aim of this study was to describe which second malignancies occur in patients with CLL, whether these malignancies are related to CLL, its treatment, or both. We also attempt to study factors predicting the development of other malignancies. PATIENTS AND METHODS: Between 1995 and 2009, six cases of CLL associated with solid tumor were diagnosed in Hematology Department of Military Hospital of Tunis. The diagnosis of CLL was made by immunophenotyping of peripheral blood circulating B cells, and the diagnosis of solid tumors was made by biopsy with anatomopathological exam and immunohistochemical study. RESULTS: The mean age of patients was 71 years. Five patients were male. The CLL was classified Stage A in one case, Stage B in three cases and Stage C in two cases. Two patients had abnormal karyotype. Three patients have not received specific treatment for their CLL. Solid tumors were represented by skin cancer in three cases, lung cancer in two cases and breast cancer in one case. The median time between diagnosis of CLL and that of solid tumor was 53 months. CONCLUSION: Patients with CLL have an increased risk of developing a second cancer. Awareness of risk factors could permit early detection.

[Atypical defibrination syndromes and acute leukemias with a t(9,22) translocation, apropos of 2 cases]. / Syndromes de défibrination atypiques et leucémies aiguës à translocation t(9,22); à propos de deux observations.

We report two cases of atypical defibrination syndromes in patients with respectively acute monoblastic leukemia (chronic myeloid leukemia initially) and acute lymphoblastic leukemia. Hemostasis studies show low fibrinogen level, elevated D-dimers, decreased alpha 2 antiplasmin and factor V, normal antithrombin III values. Plasminogen is below the normal range in one patient. Soluble complexes, which are an important argument for diagnosis of intravascular coagulation disease, are not detected in both patients. Primary or secondary hyperfibrinolysis seems also excluded since euglobulin clot lysis time was normal. Enzymatic proteolysis of fibrinogen (or fibrin) by the blast cells has been reported by some authors; this mechanism could account for the hemostasis abnormalities observed in these two patients.

[Hairy cell leukemia: report of 8 cases]. / Leucémie à tricholeucocytes à propos de 8 cas.

Hairy cell leukemia haemopathy is a rare lymphoïd haemopathy type B. 8 cases are reported and diagnosed at Hôpital Aziza Othmana over a period of 20 years between 1979 and 1999, 7 men and one women. The mean age of the patients is 51 years, with externe ages from 42 to 81 years. 4 patients consulted for an infections and, or anaemia syndrome. The disease was revealed due to the presence of an isolated splenomegaly in other cases. At the clinical examination, the spleen is hypertrophied in 7 patients out of 8. Pancytopenia is observed in 50% of the patients. Only one patient has presented a moderated hyperleukocytosis at 11,000/mm3 related to the presence of moving on tricholeukocytes. The myelogramme is pocr. It allowed to mention the diagnosis in 6 cases out of 8. Bone Marrow biopsy revealed a diffuse infiltration by TCL with a reticulinic fibrosis in all patients. 4 patients out of 8 have been splenectomized. Cytopenies have been corrected in all patients. Only one patient has been treated by alpha Interferon for 3 years with a partial hematological response. A relapse was observed once the Interferon was stopped. With the introduction of new drugs such purine analogues. The HCL treatment has been revolutionarized thanks to the improvement of the rate of complete response (from 10% to 80% of CR). If splenectomy is still observed in HCL for splenomegalic and or severe cytopenia, our findings could be improved thanks to new purine analogues.

[Long-term survival and prognostic factors in multiple myeloma treated with conventional chemotherapy. Report of 109 cases]. / Survie a long terme et facteurs pronostiques du myelome multiple traite par chimiotherapie conventionnelle. A propos de 109 cas.

Our study is retrospective. We report the results of conventional chemotherapy ins previosly untreated patients with myeloma. Survival and prognostic factors were analysed in 109 patients diagnosed from 1983 to 1992. The median age was 65 years, 87 patients (80%) were including in the stage III according the Durie Salmon staging system. The median survival time was 27 months and 10 years survival rate is 3.66%. In the univariate analysis, two prognostic variables were retained namely the hemoglobin and creatinine level. The study suggest that conventional therapy is a good treatment for old patients. However, patients younger than 55 years, must benefit from intensive chemotherapy supported by autologous bone marrow, pheripheral blood stem cells, or allogenic bone marrow transplantation. A considerable encrace in duration of remission and survival is possible.

[The 5q(-) syndrome. Report of 2 cases]. / Le syndrome 5q(-). A propos de 2 cas.

A rare and primitive myelodysplastic syndrome 5q(-) is characterised first, by the persistence of the cytogenetic anomaly 5q(-), and second, by its feminine predominance. Among 13 cases of myelodysplastic syndromes, the subject of a substantial and systematic cytogenetic medullar study (1996-1998), this paper is a case study of 2 syndromes 5q(-) diagnosed in two male patients, respectively, aged 41 and 68. The following diagnosis was made on the basis of an aregenerative macrocytic anaemia, a high platelet count, and a megakaryocytic hyperplasia, along with dysmegakaryocytopoiesis. The diagnosis of the 5q(-) syndrome was verified by cytogenetic analysis showing in one of the patients a deletion 5q(-)(q13, q33) and 5q(-)(q14, q34) with trisomy in the second one. Treatment was only limited to a blood transfusion. Subsequently one of the patients developed an advanced case of leukaemia. This paper suggests that a systematic medullar cytogenetic study must be conducted in the case of any refractory anaemia in order to identify the syndrome 5q(-) in individual cases.

[Results of a prospective protocol for the treatment of adult Hodgkin's disease]. / Resultats d'un protocole prospectif de traitement de la maladie de Hodgkin de l'adulte.

To report the results of an adapted protocol of treatment of Hodgkin disease in Tunisian patients. 70 patients (47 males and 23 females, sex-ratio = 2.04) with a mean age of 38.5 years (15 to 75) are enrolled in a therapeutic protocol to the prognostic factors and based on chemotherapy with MOPP/ABV or hybrid associated to radiotherapy. We perform an evaluation of response to chemotherapy after the 4th cycle, after the 6th cycle and then at the end of the protocol. Our population is characterized by the frequency of young patients(34% between 30 and 40 years), histologic types 2 and 3 (45 and 48%) and advanced disease with 60% of stages III and IV. After the 4th cycle, 32 patients(45%) are in complete response and 31(44%) in partial response, while 6 patients(9%) progress under chemotherapy. After 6 cycles, we observe 44 in complete response(72%) including 46% of the bad responders after 4 cycles. At the end of the protocol and on the 58 evaluable patients, 50 remain in complete response(86%). We observe 5 deaths occurred in 3 progressing patients and in 2 patients by infection after chemotherapy. 5-year actuarial and disease-free survival is 60% and 56% and median survival is 83 months. In the univariate analysis, response to chemotherapy represent the unic significant prognostic factor.