[Relapsing polychondritis, thrombosis and antiphospholipid antibodies]. / Polychondrite atrophiante, thromboses et anticorps antiphospholipides.

The authors report a case of relapsing polychondritis associated with antiphospholipid antibodies. The initial manifestations were recurrent venous thrombosis which occurred prior to the chondritis. Antiphospholipid antibodies are found in various diseases, some of which are sometimes associated to relapsing polychondritis. However, antiphospholipid-antibodies have not yet been reported in this disease.

[Varicella-zoster virus pancreatitis in hematologic diseases]. / Pancréatites zostériennes au cours des hémopathies.

We report four cases of varicella-zoster pancreatitis in immunocompromised hosts. All 4 patients presented a severe immunodeficiency because of chronic lymphoproliferative disorders (mainly lymphoma and Hodgkin disease) and long-term immunosuppressive therapy. Varicella zoster pancreatitis is a very unusual presentation of varicella-zoster infection. Few cases of pancreatitis occurring after bone marrow transplantation have been reported. All 4 patients presented with acute epigastric pain associated with transient elevation of serum amylase. The vesicular rash followed the presenting symptoms of severe abdominal pain by 8 days. This clinical presentation, occurring in immunocompromised patients, defines a set of symptoms which should lead the physician to suspect varicella-zoster pancreatitis, even in the initial absence of the characteristic skin vesicular eruption. Early institution of antiviral therapy seems mandatory.

Longitudinal melanonychia induced by hydroxyurea: four case reports and review of the literature.

Although the occurrence of skin lesions during long-term hydroxyurea therapy is well known, longitudinal melanonychia (LM) are more rarely described. In the present paper, we report four cases of LM associated with skin lesions induced by long-term daily hydroxyurea therapy (4 to 10 years), characterized by two uncommon aspects: late onset (2.5 to 5 years) and predominance of toenail involvement in three cases.

Cardiac localization of non-Hodgkin's lymphoma: two case reports and review of the literature.

Secondary non-Hodgkin's lymphoma of the heart (SNHLH) are more frequent than primitive non-Hodgkin's lymphoma and represent the third most common malignant tumour of the heart in autopsy studies. Cardiac involvement usually occurs as a late manifestation in patients with disseminated disease. Initial cardiac lymphoma, defined as cardiac involvement at initial diagnosis with concomitant extracardiac localizations, have nevertheless been reported in approximately 42 cases. The present paper concerns two patients with non-Hodgkin's B-cell lymphoma where cardiac involvement occurring 3 and 6 years after initial diagnosis constituted the unique site of relapse. These cases differ from previous reports of the literature by the predominance of extranodal localizations at initial diagnosis and the late onset of cardiac involvement. Clinical and radiological findings were otherwise in accordance with those usually described in such patients. Transthoracic echocardiography revealed the cardiac tumour in the first case, but in the second case transoesophageal echocardiography and magnetic resonance imaging (MRI) were required to demonstrate its presence. As in most reports, the site of tumour involvement was the right cardiac cavity and histology showed high grade B-cell non-Hodgkin's lymphoma. Polychemotherapy, associated with radiotherapy in the second case, led to partial or complete remission of the cardiac tumour without recurrence within the months of follow-up, although both patients died of their disease within one year.

Hodgkin's and non-Hodgkin's hepatic lymphoma: sonographic findings.

The sonographic studies of 72 patients with pathologically proven Hodgkin's or non-Hodgkin's hepatic lymphoma were retrospectively reviewed. Sixty-eight patients (94%) had secondary hepatic lymphoma (nine of them had AIDS-related lymphoma) and four patients (6%) had primary lymphoma of the liver. Forty-six of 72 patients (64%) had diffuse hepatic involvement, and 26 of 72 patients (36%) had focal liver lesions as demonstrated by sonography. Four patterns of disease were identified: (a) hepatomegaly was depicted by sonography in 26 of the 59 patients with secondary hepatic lymphoma not related to AIDS, in two of the nine patients with AIDS-related secondary hepatic lymphoma, and in one of the four patients with primary hepatic lymphoma; (b) multiple rounded well-delineated hypoechoic liver lesions were found in 22 of the 68 patients with secondary hepatic lymphoma; (c) a large heterogeneous echoic mass, which was an evocating clue to the diagnosis of primary lymphoma of the liver, was found in the four patients with primary lymphoma of the liver; and (d) an absence of sonographic abnormalities was found in 20 of the 59 patients with secondary lymphoma not related to AIDS. Liver involvement with lymphoma should be considered in any patient who develops multiple homogeneous hypoechoic liver masses, even in the absence of known underlying lymphomatous disease.

Ascites revealing peritoneal and hepatic extramedullary hematopoiesis with peliosis in agnogenic myeloid metaplasia: case report and review of the literature.

A 61-year-old man presented with ascites in the course of agnogenic myeloid metaplasia (AMM). Ascitic fluid was exudative and contained mature and immature leukocytes, erythroid cells, and megakaryocytes as observed on a bone marrow smear. Peritoneal biopsy showed myeloid metaplasia, and liver biopsy revealed intrasinusoidal myeloid metaplasia and peliosis. Ascites cleared after abdominal radiotherapy but treatment resulted in transient aplasia. Subsequently, portal hypertension was demonstrated by hepatic transjugular catheterization. Complications of splenomegaly led to splenectomy and splenorenal shunt followed by fatal acute hepatitis and septic shock. A review of the literature and an analysis of mechanisms of ascites occurring in AMM, especially peritoneal implants of myeloid tissue and occurrence of peliosis in myeloproliferative disorders, are presented.

Granulopoietic differentiation in long-term bone marrow cultures from children with congenital neutropenia.

The capacity of granulopoietic precursor cells (CFU-GM) to differentiate in vitro was evaluated in five children with congenital neutropenia using short-term colony assays and long-term marrow cultures. In all five children, methylcellulose assays revealed normal numbers of CFU-GM, which displayed an appropriate response to various sources of GM-CSF and differentiated up to the polymorphonuclear leukocyte state (PMN). In contrast, neutrophil PMN were not observed in long-term bone marrow cultures from three patients, despite a normal production of CFU-GM, myeloblasts, and promyelocytes during the 5-6 week culture period. Thus, in these patients, the characteristic "block" in granulocytic maturation observed in vivo was reproduced in vitro in long-term cultures. Granulocytic differentiation proceeded normally in long-term cultures from the two other patients, thus indicating heterogeneity in the expression of the defect. These results might indicate abnormal interactions between stromal and hematopoietic cells in long-term marrow cultures from some patients with congenital neutropenia. Furthermore, our results showed some correlation between the granulocytic defect in vitro and the clinical outcome in vivo.

[Effects of mantle-field irradiation and chemotherapy on respiratory function (author's transl)]. / Conséquences de l'irradiation médiastinale et de la chimiothérapie sur la fonction respiratoire.

In 15 patients with mediastinal Hodgkin's disease treated with chemotherapy followed by mantle-field irradiation respiratory function tests performed before and after irradiation showed a decrease in vital capacity and FEV1 as compared with pretreatment values. The decrease was maximum at 4 months and regressed over one year. No significant changes were observed in blood gas measurements. Respiratory symptoms, which were frequent after irradiation, almost completely disappeared within one year. The therapeutic combination had no cumulative adverse effect on respiratory function.