[Epidemiology of monoclonal gammopathy in a general hospital and a university internal medicine department]. / Epidémiologie descriptive des gammapathies monoclonales. Expérience d'un centre hospitalier général et d'un service de médecine interne de centre hospitalier et universitaire.

PURPOSE: Monoclonal gammopathy are common in the general population. We describe biological features and etiology of monoclonal gammopathy diagnosed during more than a ten year period in the Internal Medicine Department of Rennes University Hospital and in all the medical departments of General Hospital of Blois. METHODS: Patients were identified by immunofixation registry of Biochemistry Laboratories in both hospital (from 1990 in Rennes and from 1980 in Blois). RESULTS: Internal Medicine Department of Rennes University Hospital: 1051 monoclonal gammapathies were identified: 514 men and 537 women. Median age was 71. Isotypes repartition was: IgG 42.8% (450 cases), IgM 31.9% (335), IgA 8.9% (94) biclonal gammopathy 9.8% (103). Sixty-nine monoclonal light chains (6.6%) were identified. Median concentration of monoclonal protein was 14 g/l (1.8-104.4). All department of General Hospital of Blois: 1282 monoclonal gammapathies were identified: 700 men and 582 women. Median age was 79. Isotypes repartition was: IgG 59.7% (765 cases), IgM 27.5% (329), IgA 11.8% (151). Thirty-four monoclonal light chains (2.7%) were identified. Median concentration of monoclonal protein was 5.6 g/l (0.5-96.6). Most frequent diagnosis were: monoclonal gammopathy of undetermined significance or MGUS (77.6% in Blois and 64.1% in Rennes), multiple myeloma (11.9% and 12.7%), Waldenström's macroglobulinemia (4.4% and 8.7%). CONCLUSION: Monoclonal gammopathy are common in clinical practice. MGUS account for more than 60% of monoclonal gammopathy. Given their frequency, diagnostic and follow-up strategies must be costless and simple.

A randomized trial of amsacrine and rubidazone in 39 patients with acute promyelocytic leukemia.

Thirty-nine patients with untreated acute promyelocytic leukemia (APL) were randomly allocated to receive rubidazone (zorubicin) 200 mg/m2/d, days 1 to 4 plus cytarabine (Ara C) 200 mg/m2/d, days 1 to 7 (arm A, 21 patients), or amsacrine (Amsa) 150 mg/m2/d, days 1 to 4 plus Ara C 200 mg/m2/d, days 1 to 7 (arm B, 18 patients). Prophylaxis of disseminated intravascular coagulation was made by platelet transfusions and heparin. In case of leukemic resistance, patients received a second course with 2 days of rubidazone (arm A) or Amsa (arm B) and 3 days of Ara C. Patients who achieved complete remission (CR) received three consolidation courses with the two drugs used for induction and maintenance therapy for 3 years. Two patients in arm A and one in arm B were allografted in first CR. Initial characteristics were similar in both arms. In arm A, 18 patients (86%) reached CR, two had hypoplastic death, and one had leukemic resistance after two courses. In arm B, 12 patients (66%) achieved CR, two had early death (CNS bleeding, one case; ventricular fibrillation, one case), and four had resistant leukemia after two courses. The difference in CR rate between the two arms was not significant. In arm A, disease-free survival (DFS) showed a plateau at 54.3% after 34 months (95% confidence interval [CI], 32.1% to 74.9%), with eight CRs longer than 34 months. In arm B, DFS was significantly shorter (P less than .03), showing a plateau at 16.7% after 38 months (95% confidence interval, 4.7% to 44.6%), and only two prolonged CRs were seen. The difference in DFS remained significant after censoring allografted patients and patients who died in CR (one in arm A, two in arm B). Our results suggest that Amsa-Ara C combinations may be inferior to anthracycline-Ara C combinations in the treatment of APL, because they seem to provide shorter DFS and, possibly, a higher incidence of initial leukemic resistance. However, studies with larger numbers of patients are required.

[Gougerot-Sjögren syndrome and malignant lymphoproliferative syndromes]. / Syndrome de Gougerot-Sjögren et syndromes lymphoprolifératifs malins.

INTRODUCTION: This review is aimed at defining the frequency, anatomical and clinical presentation, pathogenesis, predictive factors and treatment of malignant lymphoproliferative diseases occurring in the course of Sjögren's syndrome. CURRENT KNOWLEDGE AND KEY POINTS: The frequency of non-Hodgkin's lymphoma (NHL) is estimated to be about 7%. Other malignant lymphoproliferative diseases (Waldenstrom's macroglobulinemia, multiple myeloma, Hodgkin's disease) are rarely observed. NHL is most frequently extranodal (affecting the salivary glands, stomach, lung, etc) in low grade malignancy (MALT lymphoma [mucosa associated lymphoid tissue]). The pathogenesis of NHL in Sjögren's syndrome is a multi-step process, including B cell monoclonal proliferation, oncogenic and/or infectious agents, and/or cytokines. Various predictive factors such as persistent enlargment of parotid glands, adenopathy, splenomegaly, mixed cryoglobulinemia, monoclonal gammopathy, suggest potential lymphoma evolution. The treatment of Sjögren's syndrome-associated NHL depends on the type of lymphoma. Moreover, in patients with low-grade lymphoma therapeutical strategies varies according to the stage and evolution of the disease. FUTURE PROSPECTS AND PROJECTS: Future prospective longitudinal studies should permit to define the best criteria for malignant transformation and to improve therapeutical strategies.

[Human babesiosis in 1997]. / La babésiose humaine en 1997.

Human babesiosis is a rare parasitic infection caused by an haemoprotozoan of genus Babesia, transmitted by tick bites. In Europe, the most severe cases are observed in asplenic patients infected by Babesia divergens. Acute intravascular haemolytic syndrome appears rapidly and is responsible for renal failure and is life threatening. Most of the cases have been reported in France. In contrast, Babesia microti babesiosis observed in the United States are less severe than Babesia divergens cases. The major problem raised by babesiosis is the rapidity of the clinical and biological diagnosis. The currently recommended treatment of severe cases consists of a blood exchange followed by clindamycine intraveine use, 25 mg/kg/d in three treatments, until disappearance of parasitemia. Prophylaxis against tick bites should be recommended for asplenic individuals.

[Felty's syndrome: retrospective study of 12 cases]. / Syndrome de Felty: étude rétrospective de 12 cas.

A retrospective study of twelve cases of Felty's syndrome was performed. The main points of this syndrome (clinical presentation, physiopathology, complications, treatment) are described.

[Triclonal gammopathy and malignant immunoproliferative syndrome]. / Gammapathie triclonale et syndrome immunoprolifératif malin.

Three distinct monoclonal gammopathies were identified in the serum of a 79 year-old man. In 1972 he presented with Waldenström's macroglobulinemia IgM Kappa. Twenty years later multiple myeloma was diagnoses. Serum protein electrophoresis performed at this time showed three monoclonal bands. Immunofixation identified these bands as monoclonal IgM kappa, IgG kappa and IgA kappa. Twenty-six cases of triclonal gammopathies were previously reported. Sixteen cases were associated with malignant immuno-proliferative diseases (non-hodgkin lymphoma, Waldenström's macroglobulinemia, multiple myeloma); five cases with non-hematologic diseases; three cases were of undetermined significance. The origin of three distinct monoclonal proteins may derive from three unrelated clones or alternatively from a single clone in which an isotype switch has occurred.

[Cryptococcal meningitis associated with chronic lymphocytic leukemia: a case report. Review of the literature]. / Cryptococcose neuroméningée et leucémie lymphoïde chronique: à propos d'une observation. Revue de la littérature.

INTRODUCTION: The authors report the occurrence of a cryptococcal meningitis in a patient treated by corticosteroids and polychemotherapy for a chronic lymphocytic leukemia. EXEGESIS: A 63-year-old man with chronic lymphocytic leukemia was sent to hospital because of impaired condition with fever. Neurological disorders appeared. Cryptococcal meningitis was diagnosed. Under treatment, the outcome was favorable. CONCLUSION: This paper highlights the feature of this infection most likely underestimated in HIV-seronegative patients and the need to a priori consider this diagnosis.

[Follow-up of monoclonal gammopathy of undetermined significance in a population of 51 patients older than 70 years ]. / Gammapathie monoclonale de signification indéterminée: suivi évolutif de 51 patients âgés de plus de 70 ans.

UNLABELLED: Monoclonal gammopathy of undetermined significance (MGUS) are very frequent in the elderly. Its incidence is about 3% after 70 years. We have performed a retrospective study of patients aged of more than 70 years who were examined until 1995 for a MGUS. METHODS: The study included 51 patients: 24 men and 27 women. Information about evolution was obtained by medical file or by asking medical practitioner. We know the evolution of all patients until January 1st 2000. The median follow-up is 5.8 years (70 months). RESULTS: The MGUS remained stable for 34 patients (67%) with a median follow-up of 83 months (12 to 180 months). Nine patients (17%) developed malignant transformation of MGUS (6 multiple myeloma, 2 Waldenström macroglobulinemia, 1 malignant lymphoma). Eight (15%) developed a cancer. Twelve died without evidence of multiple myeloma or related disorder. The actuarial probability of malignant transformation at 5 years was 12%. CONCLUSION: The risk of malignant transformation of MGUS doesn't decrease in the elderly. A regular and prolonged follow-up is necessary.

[Diagnostic splenectomy. Apropos of 38 cases]. / La splénectomie à visée diagnostique. A propos de trente-huit cas.

The authors report a retrospective study of 38 splenectomies performed as a diagnostic procedure. In the first group, the diagnosis was made by surgery: 27 cases (71.1%) included the usual large number of lymphomas. In the second group, there was no conclusion: 11 cases (28.9%), patients were younger and the spleen was lighter with a statistical significance (p less than 0.006 and p less than 0.003). Three of the 11 patients in the second group subsequently developed a malignant blood disease. Morbidity was high: out of 38 cases, 14 complications occurred in 11 patients (28.9%). One patient died on the fifty fifth postoperative day. The authors consider that splenectomy for undiagnosed splenomegaly is an effective procedure for diagnosing lymphoma, but not without risk. Indications for surgery must be carefully evaluated. It is more questionable in young, asymptomatic patients or in the case of moderate splenomegaly. After surgery, with no histologic diagnosis only a long-term survey will differentiate patients with malignant blood diseases from patients with idiopathic splenomegaly.

[Pleural involvement of myeloma. Apropos of 2 cases]. / Atteinte pleurale du myélome. A propos de deux observations.

The authors report two cases of myelomatosis localised to the pleura, one of which was associated with an adenocarcinoma. Pleural effusions are relatively rare during the course of multiple myeloma and most often occur with non-specific disorders of the disease. The myelomatous origin of a pleural effusion can only be made by analysis of the pleural fluid and should be recognised early enough to enable aggressive treatment to be instituted even if the prognosis associated with such a localisation is very poor.

[Secondary paraneoplastic polycythemia of testicular cancer]. / Polyglobulie secondaire paranéoplasique d'un cancer testiculaire.

The authors report the case of a 41-year old patient with polycythaemia and a mixed testicular tumour (seminoma with choriocarcinomatous contingent). The demonstration of a raised erythropoietin level in the tumor tissue with a high gradient in relation to the serum argues in favour of secondary paraneoplastic polycythaemia. With a follow-up of 5 years, the patient is still in complete remission from his two diseases. This case may represent the first case of testicular tumour responsible for secondary polycythaemia.

[Splenectomy for massive splenomegaly. Apropos of 36 cases]. / Splénectomie pour splénomégalie massive. A propos de 36 observations.

Thirty-six splenectomies for splenomegaly exceeding 1000 grams are reported. The enlargement of the spleen most often was related with a malignant disease (n = 32). Cytopenia was the main indication in 14 cases, and splenectomy was contemplated for diagnosis (n = 12), initial treatment (n = 6), or due to pain (n = 3) or spontaneous rupture (n = 1). Ten patients (27.8%) had an associated surgical treatment. One patient died postoperatively (2.8%) and 12 patients presented with 14 complications (33%) usually with a rapidly favorable evolution. In 11 of 12 cases (91.6%), the operation allowed establishing the diagnosis in cases of splenomegaly with an unknown origin. Lastly, it was always effective to relieve pain and in most cases improved cytopenia. The authors conclude that the patients with massive splenomegaly are improved by splenectomy, although it most often is merely a palliative treatment in cases of malignant hemopathy.

[Splenectomy in the treatment of idiopathic thrombopenic purpura in adults. Apropos of 43 cases]. / La splénectomie dans le traitement du purpura thrombopénique idiopathique de l'adulte. A propos de 43 cas.

Case reports of 43 patients with idiopathic thrombocytopenic purpura (ITP) operated upon from 1975 up to the present were analyzed. After revision of therapeutic indications the predictive prognostic factors for splenectomy, essentially the initial response to corticotherapy, were studied. Findings indicated that splenectomy is effective treatment for ITP since permanent total remissions were obtained in 76% with a morbidity of 16% and a mortality of 2%. Emphasis is placed on the need for minute pre-, intra- and post-operative precautions.