Similar cytogenetic abnormalities in two cases of plasma cell leukemia.

Plasma cell leukemia (PCL) is a very rare disease. We report two cases of PCL with complex chromosomal abnormalities: long arm trisomy and short arm partial monosomy of chromosome 1, a marker derived from chromosome 8, and monosomy 13 were found in both cases; other additional chromosome abnormalities were also present in each case. Bastard et al. reports two similar cases in this issue. Cytogenetic studies in PCL have seldom been reported in the literature: chromosomal abnormalities are most often complex: chromosomes 1, 8, 11, 13, and 14 are those most frequently involved. Such cytogenetic findings are observed in advanced multiple myeloma. Cytogenetic, clinical, and immunological findings observed in PCL and the advanced stage of multiple myeloma are arguments for the single origin of pathogenesis.

Gastric adenocarcinoma in a patient with X-linked agammaglobulinaemia.

Patients with primary immunodeficiencies are at high risk for developing haematological malignancies and, to a lesser degree, carcinoma. We report a patient with ascertained X-linked agammaglobulinaemia who developed a gastric carcinoma involving the distal part of the stomach associated with chronic atrophic gastritis and intestinal metaplasia. These latter conditions are considered to be precursor conditions and the role of chronic infections is likely. Patients with X-linked agammaglobulinaemia, as with other primary immunodeficiencies, could benefit from regular gastrointestinal evaluation, leading to early diagnosis and treatment of carcinoma.

Rheumatoid nodules indicating seronegative rheumatoid arthritis in a patient with gout.

The association of gout and rheumatoid arthritis is rare. We report the case of a patient with gout who presented with rheumatoid nodules indicating seronegative rheumatoid arthritis.

[Hepatitis C epidemiology at a general hospital center. Management and natural history as a function of the manner of identification]. / Epidémiologie de l'hépatite C dans un centre hospitalier général. Prise en charge et histoire naturelle en fonction du mode de découverte.

OBJECTIVES: To study the epidemiological characteristics of patients with chronic hepatitis C virus followed in a primary referral hospital and the clinical influence of "systematic screening" defined as the screening of patients without symptoms and with known risk factors of hepatitis C (past transfusion, past or present intravenous drug use, haemodialysis) on the natural history and treatment of chronic hepatitis C virus. METHODS: The files of 311 consecutive patients who screened positive for anti-hepatitis C virus and were seen at the primary referral hospital, Creil, from January 1992 to February 1996, were analyzed. RESULTS: Patients who underwent "systematic screening" were younger with a shorter duration of infection. They were more often intravenous drug addicts and had lower alanine aminotransferase activity and Knodell scores than patients who underwent screening during "a diagnostic procedure", because of symptoms and/or abnormal liver biochemistry. Increased age at contamination and alcohol consumption of more than 40 g per day was associated with an increased risk of cirrhosis while patients who underwent "systematic screening" had a lower risk of cirrhosis and higher survival rate. Interferon therapy was attempted less often in anti-hepatitis C virus positive patients from "systematic screening" programs. CONCLUSIONS: Anti-hepatitis C virus positive patients from "systematic screening" programs had a benign disease and were rarely treated with interferon compared to anti-hepatitis C virus positive patients diagnosed during a "diagnostic procedure".

[Spontaneous septic arthritis in disseminated lupus erythematosus]. / Arthrite septique spontanée au cours du lupus érythémateux disséminé

The authors report the case of a young 17 year-old girl with acute systemic lupus erythematosus who presented with purulent arthritis due to Group A hemolytic streptococcus in the knee, and perhaps other joints. She had not yet received any treatment. The purulent arthritis was cured by antibiotics. In spite of corticosteroids and immuno-suppressive agents (Chlorambucil), the patient died one year later. Spontaneous purulent arthritis is rare during systemic lupus erythematosus. We found only 8 other cases in the world literature. Contrary to our case, these were patients already treated with corticosteroid or immuno-suppressive agents. In our patient, the absence of previous treatment permitted us to incriminate the lupus itself in the onset of this infection. The conditions of onset of infection during lupus erythematosus are discussed.

[Myelomatous G-kappa immunoglobulin with antistreptolysin O activity]. / Immunoglobuline G-Kappa myélomateuse à activité antistreptolysine O

A new case of myeloma with antistreptolysin O activity is described. The myeloma protein is Kappa IgG. By precipitation of this myeloma IgG with anti-Kappa antiserum, the antistreptolysin O activity is completely inhibited. For the antibody activities of the myeloma protein described in the litterature, the antistreptolysin O activity is the most frequently found. With this 19th case the authors discuss the fundamental problems of the antibody activities of myeloma protein.

Early ritonavir-induced maculopapular eruption.

We report 2 cases of maculopapular eruption and fever in patients infected with human immunodeficiency virus (HIV) on the 2nd day of first administration of ritonavir, a protease inhibitor. In the 1st patient, clinical improvement occurred despite continuation of therapy, and in the 2nd the treatment was stopped with remission and rechallenge resulting in recurrence. Ritonavir should be added to the list of drugs that can induce adverse cutaneous reactions in HIV patients.

[Cases of acute leukaemia following immunosuppressive therapy for disseminated sclerosis and for Behçet's syndrome (author's transl)]. / Leucémies aiguës survenues au décours d'une maladie de Bechçet et d'une sclérose en plaques traitées par immunosuppresseurs.

On the basis of two personal cases of acute leukaemia occurring following immunosuppressive therapy for disseminated sclerosis and for Behçet's syndrome, the literature is reviewed. Thirty three similar detailed cases were collected. They are characterised in general by the prolonged use of immunosuppression. The acute leukaemia affected the granulocyte series in the majority of cases. In 30%; the leukaemia was preceded by a phase of several months of preleukaemic type dysmyelopoiesis. The mechanisms of action explaining this carcinogenic risk in a general way are multiple: the role of marrow aplasia, chromosomal abnormalities, the activation of a leukaemogenic virus, the role of immunodepression and that of repeated antigenic stimulation may all be discussed. The risks associated with such treatment should thus be borne in mind when evaluating its indications.

[Acute granulous leukemia in multiple myeloma. Apropos of 5 cases]. / Leucémie aiguë granuleuse au cours du myélome multiple. A propos de 5 observations.

The authors report five cases of acute granular leukemia which complicated the course of multiple myeloma treated by chemotherapy. They then review the literature, and discuss 30 cases which they found. The important point clinically is the long duration of the course (average 56 months) in multiple myeloma complicated by acute granular leukemia. Usually chemotherapy permitted a good quality remission and when the leukemia develops there is no longer any trace of myeloma. The mechanisms of onset of this type of leukemia during myeloma are discussed. They are probably complex and various but there is no doubt concerning the responsability of chemotherapy and in particular alkylating agents.

[Treatment of polycythemia. I--Using radiophosphorus with or without treatment in 483 patients over 65 years of age]. / Traitement des polyglobulies. I--Utilisation du radiophosphore avec ou sans traitement d'entretien chez 483 malades âgés du plus de 65 ans.

AIMS: To compare by a prospective study in high risk polycythemia vera (PV) patients 33P alone and 32P followed by low-dose hydroxyurea (HU) maintenance therapy. Toxicity, efficiency, and leukemogenic potential were studied. PATIENTS: 483 patients with a documented PV, aged more than 65 years at diagnosis, were included between 1980 and 1996 in a prospective study comparing 32P alone and 32P followed by low-dose HU maintenance therapy. Blood cell counts were performed every two months and a clinical evaluation by a specialist was obtained every four or six months. RESULTS: Treatments were well tolerated, but chronic leg ulcers were observed in the maintenance therapy arm. The risk of leukemia was about 15% at the 15th year in the group of patients treated by 32P alone, but reached 30% in the group receiving maintenance therapy. In both arms, there was no significant correlation between occurrence of leukemia and the total dose of 32P. There was a correlation between the leukemic risk and disease severity, estimated on the frequency of relapse. Cancer occurrence was slightly higher than expected in the maintenance arm. HU treatment did not protect against progression to myelofibrosis, probably due to the lack of maintenance of an efficient myeloid or megakaryocytic suppression. Median life-span was slightly shorter in the group receiving HU maintenance. In all cases, life-span was only one year lower than that observed in the reference population. CONCLUSION: For all these reasons, we suggest the us of 32P alone in elderly patients; complementary chemotherapy should only be prescribed in the cases with short-term relapse, and late resistance to 32P.

[Psoriatic arthritis with renal amyloïdosis. A case-report. Review of the literature (author's transl)]. / Rhumatisme psoriasique et amylose rénale. A propos d'un cas. Revue de la littérature.

A case of severe psoriatic arthritis with both spinal and peripheral joint involvement is reported. The patient, an HLA B27-positive man, was thirty-one years old at onset. Both antiinflammatory drugs and immunosuppressive agents (chlorambucil followed by azathioprine) were ineffective and the patient became bed-ridden. Thirty-three months after onset a severe nephrotic syndrome developed. Renal biopsy let to diagnosis of secondary renal amyloïdosis. The patient died five months later in spite of chemotherapy with melphalan and prednisone. Autopsy could not be performed. A review of the literature showed twelve other cases of psoriatic arthritis with secondary amyloïdosis. Men are affected more often than women. Twelve years was the average interval between onset of joint involvement and diagnosis of amyloïdosis. In three instances, however, this interval was less than thirtyeight months. Arthritis often included both spinal and peripheral joint involvement. Amyloïdosis was evidenced by a nephrotic syndrome in eight out of twelve cases. Prognosis is poor with a fourteen months average interval between onset of clinical amyloidosis and death.

[Extra skeletal Ewing's sarcoma. Report of two cases. Ultrastructural study of one case (author's transl)]. / Les sarcomes d'Ewing extra-osseux. A propos de deux observations dont l'une avec étude ultrastructurale.

The authors report two cases of extra skeletal Ewing's sarcoma. The first case concerns a 26 years old woman presenting a tumor at the level of the sacrum area, locally recurrent, metastazing to the lungs and the lumbar column, despite of radiotherapy and chemotherapy and leading to death after a course of 18 months. The second one is that of a 30 years old man bearing a tumor of the shoulder area probably already metastazed to bones, rapidly recurrent and metastazing to the lungs and cause of death after 9 months in spite of intensive therapy. About these 2 observations a review of the literature of the cases of extra skeletal Ewing's sarcoma is done. Whatever nosologic discussion it seems that Ewing's sarcoma may present essentially as a tumor of soft tissues. An ultrastructural study has been performed in the second case. The findings are similar to those reported in Ewing's sarcoma.