[Extramedullary plasmocytomas]. / Plasmocytomes extraosseux.

PURPOSE: To study the localization, treatment and prognosis of extramedullary plasmocytoma through a series of eight patients and a literature review. PATIENTS AND METHODS: Eight patients with extramedullary plasmocytoma were treated in the university's hospital of Sfax in Tunisia. The average age was 57.3 years. Female represented 75% of patients. The diagnosis of plasmocytoma was based on anatomo-pathology and immunohistochemistry of a biopsy or resected tumour. Extramedullary location was confirmed if biological and radiological exams and medullary biopsy were normal. The therapeutic decision was made after multidisciplinary meetings regarding tumour location and anterior treatment. RESULTS: Solitary extramedullary plasmocytoma was located in nasal cavity, cervical node, testis, ovary, bladder and the tongue. One patient was treated for three simultaneous locations of extramedullary plamocytoma (node, bowel, pleura) without evidence of myeloma. Radiotherapy was proposed in six cases but refused in one case (plasmocytoma of the bladder is currently receiving radiotherapy). Treatment consisted in chemotherapy in two cases. Evaluation after treatment revealed complete remission in 86% of the cases. Nodal recurrence was noted in two cases. These two patients were lost to follow up. The five other patients were in complete remission after a mean follow up of 5.7 years. No local recurrence or myeloma was noted. CONCLUSION: Extramedullary plasmocytoma is a rare affection. It can occur in any region of the body. Head and neck is most frequent localization. The treatment is irradiation or surgery in some localization. Progression to myeloma is the most important factor that influences the prognosis of the disease.

Evaluation of serum VEGF levels in untreated erythrocytosis patients.

The aim of this prospective study was to investigate the involvement of angiogenesis in the etiopathogenesis of the different classes of erythrocytosis (polycythemia vera PV, idiopathic erythrocytosis and secondary erythrocytosis). The angiogenic activity was evaluated by the assessment of the serum VEGF levels in 78 untreated erythrocytosis patients and 21 healthy subjects. The results indicated that VEGF was overproduced in advanced and untreated PV patients and at less degree, in early PV, a subgroup of idiopathic erythrocytosis, thus confirming an increased angiogenic activity. However, VEGF does not play an angiogenic role in secondary erythrocytosis.

B-cell lymphoma of the mandible.

INTRODUCTION: Primary malignant lymphoma of the mandible is rare. It is frequently mistaken for a dental lesion, delaying diagnosis. We here report a case of mandibular lymphoma and present the clinical and radiological characteristics and means of treatment of this pathology. CASE REPORT: A 17-year-old woman consulted for right facial tumefaction with 1 year's evolution. Clinical examination found a mass facing the horizontal branch of the mandible, extending to the right parotid region without cutaneous involvement. CT and MRI showed a large expansive process of mandibular origin with parotid, temporal and intracranial extension. Biopsy indicated large B-cell lymphoma. The patient showed good evolution 2 years after chemotherapy. CONCLUSION: Lymphoma is the second most frequent form of head and neck tumor, after epidermoid carcinoma. Only 0.6% of locations are mandibular, almost always consisting of B-cell lymphoma. They are often initially misdiagnosed as a dental pathology. Complete remission after chemotherapy ranges from 60 to 80% at 1 year.

Invasive aspergillosis: epidemiology and environmental study in haematology patients (Sfax, Tunisia).

Invasive aspergillosis (IA) is a major opportunistic infection in haematology patients. Preventive measures are important to control IA because diagnosis is difficult and the outcome of treatment is poor. We prospectively examined the environmental contamination by Aspergillus and other fungal species and evaluated the prevalence of invasive aspergillosis in the protect unit of haematology. A three-year prospective study (December 2004-September 2007) was carried out in the department of haematology of Hedi Chaker Hospital. Suspected invasive aspergillosis cases were reviewed and classified as proven, probable and possible invasive aspergillosis using the EORTC criteria. During the study period, we collected weekly environmental samples (patient's rooms, tables and acclimatisers) and clinical samples from each patient (nasal, expectoration and auricular). Among 105 neutropenic patients, 16 had probable and 13 had possible IA. A total of 1680 clinical samples were collected and A. flavus was most frequently isolated (79.2%). Analysis of 690 environmental samples revealed that Penicillium (44%) was the most frequent followed by Cladosporium (20%), Aspergillus spp. (18%) and Alternaria (13%). The PCR-sequencing of 30 A. flavus isolates detected from clinical and environmental samples confirmed the mycological identification. Our findings underline the importance of environmental surveillance and strict application of preventive measures.

Primary Plasmacytoma of the Testis with no Evidence of Multiple Myeloma: a New Case Report and Literature Review

Plasmacytomas of the testis are extremely rare tumours; especially when occurring in the absence of a previous or concurrent diagnosis of multiple myeloma. We report a new case of solitary testicular plasmacytoma; with immunohistochemical studies showing monoclonal cytoplasmic production of IgG lambda light chains; in a 51-year-old man who had no evidence of multiple myeloma 3 years after the orchiectomy

[Isolated extramedullary adnexal relapse of acute lymphoblastic leukemia: a case report]. / Rechute extramédullaire annexielle isolée d'une leucémie aiguë lymphoblastique : à propos d'un cas.

The occurrence of an isolated ovarian or pelvic relapse of acute lymphoblastic leukemia (ALL) in complete remission after chemotherapy has rarely been described. We report the case of a 12-year-old girl, treated for ALL, who developed an isolated left ovarian and fallopian tube localization without medullary or blood relapse 4 years after the end of the initial treatment. She presented with an isolated pelvic mass. The diagnosis was established by a CT-guided biopsy. The treatment consisted of a second course of chemotherapy and complementary surgery; a second complete remission was obtained.

[Recurrent inflammatory myofibroblastic tumor with renal, retroperitoneal and lymph node involvement]. / Tumeur myofibroblastique inflammatoire récurrente avec triple localisation, rénale, rétropéritonéale et ganglionnaire.

Inflammatory myofibroblastic tumors are uncommon and benign tumors with unknown aetiology. First reported in the lungs, the inflammatory myofibroblastic tumors have been observed in other locations, especially in the abdomen and the pelvis. We report a 14-year-old adolescent female, who presented sequentially an inflammatory pseudotumor of lymph node, the left kidney and the retroperitoneum. Extrapulmonary inflammatory myofibroblastic tumors are mesenchymal solid tumors. They are frequently circumscribed and confined to a single organ. The recurrence of some inflammatory myofibroblastic tumors and their expression of chromosomal abnormalities found in some types of lymphoma suggest that some of these lesions constitute a true neoplastic process.

[Evaluation of a prospective therapeutic protocol in adult patients with nonsevere aplastic anemia in South Tunisia]. / Evaluation d'un protocole thérapeutique prospectif des aplasies médullaires acquises non sévères de l'adulte dans le Sud tunisien.

INTRODUCTION: Immunosuppressive drugs are usually used in the treatment of acquired aplastic anemia (AAA). The aim of this study was to evaluate the efficiency of a prospective therapeutic protocol using cyclosporine and androgens in the treatment of adult patients with nonsevere AAA. METHODS: Twenty-nine patients diagnosed and treated at the University Hospital of Sfax (Tunisia), during a 10-year period (1991-2000) were included. In addition to symptomatic treatment (transfusion, antibiotics), all the patients received a specific treatment including two drugs: cyclosporine 5mg/kg per day and androgens 0,5mg/kg per day. RESULTS: The response rate at three, six and 12 months were 48, 75, and 87%, respectively. Survival rate was 52% at one year, and 37% at five and 10 years. The main toxicities were hepatic, renal, and hypertension, observed in 53, 16 and 15%, respectively. These toxicities were reversible in 65, 87 and 100% of the cases, respectively. CONCLUSION: The response and survival rates in our series are quite satisfactory when compared to those obtained with other immunosuppressive drugs (cyclosporine and antilymphocyte serum) in the literature. The addition of androgens in our patients seemed to potentiate the immunosuppression induced by ciclosporin, but secondary toxic effects were more common.

[Pediatric Hodgkin disease in south Tunisia: a study of 23 cases]. / Maladie de Hodgkin de l'enfant dans le sud tunisien: étude de 23 cas.

PURPOSE: To discuss, through a retrospective study, the epidemiologic and anatomo-clinic aspects of paediatric Hodgkin disease (HD) in south Tunisia and to study the faisability of the French protocol MDH 90 for our patients. PATIENTS AND METHODS: Between January 1995 and December 2000, we treated 23 children with HD. Initial work-up included clinical examination, radiological explorations (chest X ray, abdomen ultrasonography and computed tomography scan of chest, abdomen and pelvis), biological explorations (full blood count, erythrocyte sedimentation rate, serum albumin, liver functions tests) and a bone marrow biopsy. Treatment was inspired from the SFOP MDH 90 protocol. Overall survivals and disease free survivals were estimated with Kaplan-Meier method and compared with the log-rank test. RESULTS: Mean age was 8.6 years (4-14 years), sex-ratio was 3.6. Histological subtypes 2 and 3 had the same frequency. Localised stages (I-II) were as frequent as advanced stages (III-IV). At the end of the treatment, the 18 evaluables patients were in complete remission. The 5-year overall survival (OS) and disease free survival (DFS) were 82 and 76% respectively. Localised stages and advanced stages had the same 5-year overall survival (82%). There were no statistical differences in term of OS between the different prognosis factors studied. CONCLUSIONS: The particularity of our study was the identical frequency of the two histological subtypes 2 and 3 and of localised and advanced stages. The MDH 90 protocol is feasable for our patients with satisfactory results similar to those found in the literature.

[Myelodysplastic syndrome / acute myeloid leukemia with t(8; 21) and bundle of Auer rods in neutrophils: an unusual hemopathy]. / Syndrome myélodysplasique / leucémie aiguë avec t(8;21) et polynucléaires à corps d'Auer en fagot: une hémopathie rare de présentation inhabituelle.

We report the case of a 8-year-old girl diagnosed with myelodysplastic syndrome. This case was morphologically characterized by the presence of bundle of Auer rods in the neutrophils. The evolution of the disease was marked by a quick transformation in a acute myeloid leukaemia with t(8;21) refractory to treatment. We reviewed the literature for clinical, biological and therapeutic features of this rare childhood hemopathy.

[Monoclonal gammapathies in Tunisia: epidemiological, immunochemical and etiological analysis of 288 cases]. / Gammapathies monoclonales en Tunisie: analyse épidémiologique, immunochimique et étiologique d'une série de 288 cas.

From July 1992 to December 2000, 288 cases of monoclonal gammapathy (MG) were collected at the university hospital of Sfax. The middle age of the patients at the time of the diagnosis was 62 years and 7 months with extremes to 18 months and 99 years and median to 64 years. One hundred and eighty-two patients were men and 106 women. Among the 270 observations for which aetiology has been established, 73 were classified MG of undetermined significance (MGUS), 160 myeloma (or plasmocytoma) and 37 other malignant MG (Waldenstrom's macroglobulinemia: 13, lymphoma: 9, alpha heavy chains disease: 6, primary amyloidosis: 5, chronic lymphocytic leukaemia: 4). Rheumatological affections (19.2%), infections and renal failure (10% each), haematological and autoimmune diseases (9.6% each) were pathologies most often associated with MGUS. Agarose gel electrophoresis did not show a monoclonal peak in 16% of the cases. In the 242 patients with a peak on electrophoresis, the peak was in the beta zone in 22% of cases and in the gamma zone in 78% of cases. The IgG isotype represents more than the half of the cases of our set (51.7%). IgG is even more predominant in the MGUS group (65.8%). The IgA isotype counts for 20.8% of the cases in our set and the free light chains (kappa or lambda) for 13.6% of the cases whereas the IgM represents 8.7% only of the 288 cases of our set which involves three cases of IgD myeloma and six cases of biclonal gammapathy.