[Focus of intrathoracic extramedullary hematopoiesis. A little-known cause of posterior mediastinal tumor].
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Biomedical subjects
Publications and source records attributed to M Boiron.
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Between 1978 and 1980, 45 cases of acute monoblastic leukaemia have been diagnosed, treated and followed in our institute. Morphological diagnosis was performed according to the French-American-British classification. Tumoral syndrome (particularly extra-medullary) and hyperleucocytosis were the most striking findings at the time of diagnosis. Cytogenetic analysis performed in 31 cases before treatment has showed that abnormality of the long arm of chromosome 11 seemed to be more frequently associated with the poorly differentiated cytological subtype M5 (a). Intensive chemotherapy with zorubicin and cytosine arabinoside led to complete remission in 75% of the cases. Central nervous system prophylaxis appeared definitively useful in preventing meningeal relapse. Despite a prolongation of the median duration of complete remission which now reaches 12 months, the prognostic is still poor.
"Xantholeukemia" is a rare disease which combines a juvenile chronic granulocytic leukemia with skin xanthomas and normal serum cholesterol level. The prognosis is variable, usually lethal, sometimes chronic with possible spontaneous cure. We collected 11 patients aged 3 months to 3 years and half at diagnosis. Nine died, 7 with a terminal "blastic" marrow. Blastic crisis was sometimes present at the onset, sometimes started later, between 9 months and 9 years after the onset. One child is still alive but in blastic crisis. One young girl is hematologically normal 13 years after the onset. At the onset, she had no bad prognostic signs. And she received no treatment. Five children had "café au lait" spots, 4 of them had familial history of neurofibromatosis. This frequent association confirms a genetic predisposition. Eight patients had one or several examinations of bone marrow and blood cytogenetics. Only one had an evident clonal abnormality: monosomy 7 (45 XY, -7) in all bone marrow cells. This monosomy 7 was discovered 21 months after the onset and 3 months before the blastic crisis. No chemotherapy had been used before this karyotype. Chemotherapeutic agents were essentially corticosteroids, 6 mercaptopurine and cytarabine. They gave an imperfect result and did not influence the outcome; therefore an aggressive treatment or a bone marrow transplantation should be discussed.
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One third of all extranodal sites of non-Hodgkin's lymphomas are localized to the digestive tract. Forty-six cases with digestive tract localization were studied, of which 15 were localized forms. Histologically, 70 p. cent were diffuse lymphomas with poor prognosis. The present survival rate for localized forms following chemotherapy is 77 p. cent, which plateaux at two years, as compared to 34 p. cent for disseminated forms. Treatment of digestive tract localizations should be similar to that of nodal forms with the same histology. With the development of intensive chemotherapeutic regimens over the last few years, the cure rate is hoped to attain 65 p. cent in disseminated forms, and 87 p. cent in localized forms.
We report our experience in the treatment of meningeal relapses in acute lymphoblastic leukemia (ALL) with intraventricular chemotherapy via an Ommaya reservoir. We treated 5 children in this way with some complications secondary to the use of the reservoir: methotrexate leukoencephalopathy, bacterial meningitis, reservoir malfunction. But patient comfort homogeneous drug distribution when injected via the Ommaya reservoir and the possibility of long term meningeal remission justify the discussion of the use of an Ommaya reservoir use in meningeal relapses in ALL.
A retrospective study of 119 patients with acute promyelocytic leukemia (APL) treated with similar DNR containing regimens in reported. Antecedent of radiation exposure or cancer was found in 10 patients. At presentation hyperleucocytosis was rare (13/119 greater than 30 000/microliter); variant form was identified in 5 cases. Organomegaly was uncommon and severe metabolic abnormality was never noted at presentation. DIC was observed in 75% of pts; t (15;17) was confirmed in 25/30 pts. Complete remission (CR) rates have increased from 43% to 76% on account of improvement of supportive therapy with adequate DIC management. Addition of ARA C did not improve CR rates (72%). Surprisingly duration of CR seems related to maintenance therapy as 11/26 pts receiving 6 MP-MTX maintenance regimen were long-term survivors as compared to 1/34 comparable pts receiving cyclic monthly courses of chemotherapy.
Herpes virus hominis infection was observed in 28% (diagnosis ascertained on viral examination) to 33% (clinical suspicion) of 36 adult patients prospectively studied during remission induction chemotherapy for acute leukaemia. Symptoms are non specific so that diagnosis relies upon viral isolation. All infected patients but one survived the viral infection and the prognosis of the underlying acute leukaemia was unaffected.
From April 1972 to September 1979, 121 patients with Hodgkin's disease clinical stages IInA, IB, IIB or III successively received MOPP 6 courses in a first trial and 3 courses in a second trial, prior to extended field irradiation; 118 patients underwent surgical restaging prior to irradiation. Anatomical findings demonstrated that 3 courses of MOPP were as effective as 6 courses to treat occult splenic disease. Comparison between clinical and surgical restaging confirmed the reliability of clinical criteria of complete remission after chemotherapy. After extended field irradiation, actuarial survival and relapse-free survival at 4 years were the same whether the patients had received 6 or 3 courses of MOPP.
Twenty-three patients with advanced measurable gastric cancer were treated with FAM-chlorozotocin in an attempt to demonstrate improvement in rate and duration of response over FAM (5-fluorouracil, Adriamycin and mitomycin C). Six (26%) partial responses were recorded with a median duration of two months. FAM-chlorozotocin was well tolerated with moderate myelosuppression as the major dose-limiting toxicity; the leukocyte nadir was 3.0 X 10(3)/mm3 (range, 0.9-6.0) and the platelet nadir was 100 X 10(3)/mm3 (range, 45-100). The authors were unable to find significant differences in prognostic factors such as performance status, sites of metastatic disease, intensity of therapy to account for the discrepancy in response rates between FAM (42%) and FAM-chlorozotocin (26%).
Experiments in the unanesthetized rats were made to examine the possibilities of relations between visceral and abdominal sites of electrodes implantation. From gastric duodenal ileal electrodes wire, relations may be observed with the surface records with the following limitation: in the frequencies greater than or equal to 200 cycles/mn ("A" range) the spiking visceral activity is never found from surface derivation where the EKG is frequently present. In the ranging of 60 to 80 cycles/mn correspondences of the signals are in relation with respiratory transmission. The slower frequencies from surface activity (longlasting RC time constant of 6 s are used) concern duodenal (38,8 +/- 0,98 cycles/mn) and ileal (28,6 +/- 1,9 cycles/mn) slow waves activities may be recognized in the sequences during several minutes in alternation with the C type activities. In such conditions of typical correspondences, it may be noticed modifications of signal during the viscero-parietal transmission: attenuation (averaging 0,7); dephasage of 5 to 12 seconds (swing to strong capacitive component acting as an active filter) and mixing with others activities not be identified. The methodological interest for a non invasive characterization of visceral physiological states is discussed.
Herpes virus hominis infection was observed in 28% (diagnosis assessed on viral examination) to 33% (clinical suspicion) of 36 adult patients prospectively studied during remission induction chemotherapy for acute leukaemia. Symptoms are non specific so that diagnosis relies upon viral isolation. All infected patients but one survived the viral infection and the prognosis of the underlying acute leukaemia was unaffected.
The authors reported the effect of high dose intravenous immunoglobulin therapy in 18 patients with chronic autoimmune thrombocytopenic purpura. A good but transitory response was observed in 60% of the cases. The intensity of the response was variable, the duration was between 11 and 26 days. The mechanism of the effect of this therapy is poorly understood. Since this therapy is expensive and the response inconsistent and transitory, the indications are limited.
The results of a cytogenetic study on 240 acute nonlymphocytic leukemia patients (187 adults and 53 children) were classified in NN (normal), AN (abnormal and normal) and AA (abnormal). Pronostic value of the classification was presented. A higher proportion of complete remission failures was observed in chromosomally abnormal patients (AN and AA). Survival of patients with complete remission was significantly shorter in AN and AA patients than in NN patients. An excess of constitutional chromosome abnormalities was observed in children.
Chemotherapy-induced alopecia observed in cancer patients can now be prevented by a simple, effective, inexpensive and well tolerated procedure: scalp hypothermia. Refrigeration is obtained by placing on the scalp two bags filled with crushed ice 15 minutes before, and removing them 15 minutes after intravenous injection of antineoplastic drugs. Only patients treated with drug combinations that are rapidly administered (into the giving-set tube or by i.v. infusion lasting less than 60 minutes) seem to benefit from scalp hypothermia. The fact that good results were obtained with those drugs (adriamycin, cyclophosphamide, 5-fluorouracil, methotrexate, vincristine) and modes of administration that are most commonly used in women with breast cancer or ovarian cancer makes this procedure extremely interesting.
Adjuvant chemotherapy trials in gastric and colorectal carcinomas are reviewed and no clear benefit from such treatment could be observed. To improve existing chemotherapy regimens, several pilot studies were performed in 95 gastric cancer and 85 colorectal carcinomas patients. Response rate for gastric carcinomas using the mitomycin C plus adriamycin plus 5-fluorouracil (5-FU) (FAM) regimen was 40% and was not improved by the addition of nitrosoureas. In colorectal carcinomas none of the combinations used gave better results than 5-FU alone. Fourteen patients with esophageal carcinoma were treated with combination CDDP and the response rate was as high as 50%.
The kinetics of methotrexate were studied in a group of 59 patients after infusion of high doses. From the triexponential analysis of the serum concentration curve, rate constants and relative concentrations in non-circulating pools were calculated, using a linear 'mammillary' three-compartment model. No significant variation as a function of dose (300-6,000 mg/m2) was observed. In circumstances the rapidly exchanging pool was a reflection of the circulating pool, suggesting that it is an extravascular, protein-bound pool of methotrexate. Considerable variations of the relative concentration and rate constants of the slowly exchanging pool were observed to be a function of the duration of methotrexate infusion, the presence of other drugs, and the patient's age. If this compartment is assumed to include the target cells for methotrexate, then the large variations observed could be responsible for individual differences in toxicity and/or effectiveness.