Biomedical subjects
Publications and source records attributed to N Chelloul.
A 63 year-old patient with pancytopenia aplastic bone marrow of several years duration and incomplete response to anabolic steroids is reported. The bone marrow biopsy reveals within lymphoid areas, a refringent pigment proved by microanalysis to be a gold salt.
The authors describe the fibro-granulomatous lesions with scar formation on a splenectomy specimen removed for familial spherocytosis, eight weeks after the onset of cat scratch disease. This original observation, apparently unique in the literature, is in favour of the blood spread of the agent responsible for benign inoculation lymphoreticulosis, and suggests a pathogenic theory for the unusual visceral manifestations.
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The authors report 5 cases and study the thoracic and abdominal lesions of actinomycosis. This is a rare bacterial disease but not exceptional, discovered usually by chance on operative specimen on autopsy. The clinical and bacteriological diagnosis is very difficult although the lesions are characteristic. Thus it is always necessary to carry out biopsy and seek sulphur granules in the actinomycotic follicle. The differential diagnosis is usually easy with granulomycoses, but it is sometimes more difficult to distinguish the disease from nocardioses.
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Neoplastic cells from 9 patients affected with a "histiocytic" lymphoma were studied with 5 membrane markers of B or T lymphocytes. In 2 patients a monoclonal B-cell proliferation was found; they had been affected previously with well documented B-cell proliferations: chronic lymphocytic leukemia or Waldenström's macroglobulinemia. The blast cells of 2 other patients had T-cell features; in a fifth case, the abnormal cells carried only a strong receptor for the Fc fragment of IgG, which suggested their truly monocytic origin. In 4 patients, the cells had no detectable surface markers. These findings demonstrated that this group of lymphomas is heterogenous, that the term "histiocytic" appears to be wrong in most instances, and that the cellular origin of the malignant cells frequently remains unidentified and thus prevents a satisfactory new classification.
The authors reported 12 cases of patients with cutaneous involvment associated with the presence of Sezary cells in the peripheral blood and specific lymph node involvment. They classify these cases as partial, early segmentary types, advanced types and diffuse types. either leukemic or sarcomatous. This study, once again, suggests the possibility that the Sezary syndrome and mycosis fu ngoïdes are different expressions, either predominantly leukemic, or predominantly sarcomatous, of the same chronic malignant hemopathy of "T" lymphocytes.
The study of the evolution of 168 cases of idiopathic myeloid splenomegaly allowed to point out the prognostic value of the clinical hematologic, isotopic and radiological parameters. The correlation of these data with the histological type of the osteomedullary lesions at the time of the diagnosis, allows to confirm the long survival of the hyperplastic formes (type I) and the more reserved prognosis in advanced myelofibrosis (type II and III together). Indeed, median survival is of 82 months for type I, and 60 months for type II and type III together. It seems advice to gather the two last types and so, to compare two anatomoclinical entities : one is characterized by hyperplastic marrow with reticulinic fibrosis, usually associated with enlarged spleen and sometimes even with increase red blood cells volume but without radiological bone lesions; the other one is characterized by a marrow of middle importance or marked marrow depletion, fibrosis, with or not osteosclerosis, and shows moderate or marked radiological lesions (respectively 16,9% of the cases) and, often, pancytopenia.
This study of the history of 117 cases of agnogenic myeloid metaplasia with myelofibrosis underlines the importance of anatomo-pathological and radiological parameters for prognosis. Histological staging of bone and bone marrow lesions at time of diagnosis enables one to confirm long survival of cases with hyperplasia (Group I) and more severe prognosis for advanced myelofibrosis (that is both groups II and III considered together) but does not enable to establish a significative difference between medium survival of cach group II and III considered separately. Those medium survivals estimated are the following: 82 months for group I; 60 months for both groups II and III. Thus, it is logical to consider together these two groups among which one can distinguish two anatomoclinical forms. One is characterized by bone marrow hyperplasia with reticulin myelofibrosis and is usually associated with large spleen and even in some cases with polycythemia but without radiologic bone lesions; the other is characterized by bone marrow of medium or hypoplasic constitution fibrosis, with or without osteosclerosis and with radiologic bone lesions of mode-rate or important extension (respectively 16.9 p. cent of cases) and is usually associated with pancytopenia.
A malignant hepatoma occurred in a 12-year-old girl who eight years previously had developed an acute lymphoblastic leukaemia which for eight years had been in complete haematological remission. Fourteen months after the last re-induction treatment period had been discontinued, but while on methotrexate and 6-mercaptopurine maintenance, a hepatocellular liver carcinoma developed of which the patient died after a fulminating course, still in complete haematological remission. As far as is known, no direct carcinogenic effect can be ascribed to the two antimetabolites, but it must be assumed that these two drugs, taken by the patient for over seven years, led to cirrhosis of the liver whose malignant transformation was significantly influenced by the immunosuppressive effects of methotrexate and 6-mercaptopurine, given as maintenance therapy according to protocol 02 LA 64, Paris.
The diagnosis of myelofibrosis depends on three fundamental criteria. Myeloid metaplasia of the liver and spleen, absence of Philadelphia chromosome and a variable degree of myelofibrosis. It is thus necessary to have available bone and marrow biopsies to make the diagnosis. The analysis of elementary lesions, together with their grouping, was at the origin of various pathological classifications, including those of Hickling and Jacquillat, Chom and Ripault. The latter, adopted by most authors, distinguishes three histological types, depending on the degree of myelofibrosis and bony sclerosis. They are usually considered as successive stages of the disease.
204 patients with Hodgkin's disease not previously treated were given vincristine, chlormethine, procarbazine and prednisone (protocol H2-65) for six months, followed by monthly vinblastine injections. Additional prophylactic radiotherapy was given to 50 non-randomised patients. Three-monthly reinduction treatment was administered to a further 50 patients during the year following the original induction cure, afterwards only every six months, and no treatment after four years. Among 109 patients in stage III complete remission occurred in 53% and partial (incomplete) remission in 36%. Among the 58 patients in stage IV, 21 had complete and one incomplete remission. Among 37 patients in stages I and II, 33 went into remission. The remission curves reached a plateau in the 42nd month in 74 plus or minus 6% of those in complete and 56 plus or minus 8% of those in incomplete remission (P less than 0.03). Increasing age had an unfavourable prognosis: the more progressed the anatomical stage the less favourable the prognosis. Surprisingly, stage IV (lymphocyte-poor) had a better prognosis on polychemotherapy than other histological forms. Signs of clinical activity did not influence prognosis, but biochemical signs of activity are unfavourable for remission duration (P less than 0.01). Patients who also had radiotherapy had longer remissions (P less than 0.01). Exacerbations occurred in 38, never beyond the 42nd month. Recurrence occurred in 21 of them in the dame lymphatic region as was affected at the beginning of the disease, while in the other 17 patients an extralymphatic episode occurred. There was a positive correlation between histological form and type of recurrence.
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Two hundred and forty-four previously untreated patients seen since 1964 in the department of haematology at Saint-Louis hospital were analysed. Clinical data included results of initial work-up and prognosis evaluated by survival rate matched with principal variables: age, sex, histopathology, staging, incidence of clinical and biological systemic symptoms. Immunological data included results of systematic studies made in each category of non-Hodgkin's lymphomata. Hyperbasophilic lymphoma, a special group recently characterized, is discussed.
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