Philadelphia chromosome-negative progenitors in chronic granulocytic leukemia.
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Biomedical subjects
Publications and source records attributed to J Reiffers.
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Peripheral nerve biopsies from two patients with chronic sensorimotor neuropathy were studied. The first case was a non-Hodgkin malignant lymphoma and did not show any dysglobulinemia. The second case had a benign monoclonal gammopathy IgG, Lambda type. Direct immunofluorescence showed no deposits in the first case and slight deposits of anti IgG sera on a few myelinated fibers in the second case. There were numerous fibers showing uncompacted myelin lamellae, 7% in the first case and 4% in the second case. Some of these fibers had axons containing more tubules than filaments. The very few cases reported on neuropathies showing that uncompacted myelin lamellae were frequently associated with dysglobulinemic neuropathy. However, this ultrastructural abnormality of the myelin sheath can be observed without any dysglobulinemia.
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Report of 2 patients suffering from sensory peripheral neuropathy and multiple myeloma. In 1 case, peripheral neuropathy occurred several months before the appearance of multiple myeloma. A peripheral nerve biopsy exhibited numerous amyloid deposits within the endoneurium. Amyloid deposits have been estimated to occur in 15% of cases of multiple myeloma, but only 4 other detailed observations of amyloid deposits within the endoneurium have been reported in the literature. Other mechanisms might be involved since most peripheral neuropathies associated with multiple myeloma do not exhibit any amyloid deposits.
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Labelling platelets with 111 Indium-oxine has advantages over the conventional 51 Chromium method: labelling is more efficient and the radiations emitted almost exclusively consist of gamma-rays. Owing to these advantages, autologous platelets can be used for kinetic studies in patients with idiopathic thrombocytopenic purpura, even when thrombocytopenia is severe. 111 Indium labelling also provides accurate information on the sites of platelet destruction, which may help to predict the patient's response to splenectomy.
In adults, acute lymphoblastic leukemia (ALL) is less frequent and less clearly defined than in children. A retrospective study of fifty adult patients with ALL was carried out. Findings were similar to those reported in previous studies. Complete remission was achieved in 86% of patients. This rate is similar to that recorded in children with ALL. Median survival (twenty months) was shorter than in childhood because of frequent relapses. Various factors of prognostic value are discussed. Problems encountered in attempting immunological classification are studied. Prognosis seems to be related to the intensiveness of induction therapy and the choice of maintenance therapy regimen.
Bone marrow karyotypes of 78 adult patients with acute myelogenous leukemia (AML) were studied at the time of diagnosis, with special reference to the Sakurai and Sandberg classification (NN, karyotype completely normal; AN, mixture of normal and abnormal metaphases in the karyotype; AA, totally abnormal karyotype). The results showed no difference in complete remission rate (CR) or in survival time between the NN and the AN groups, but highly significant differences between the AA group and the NN and AN groups (whether taken together or separately). When studying the relationship between age and survival time, we found that 59 years of age was a frontier between two homogeneous groups having quite different prognoses. The NN/AN/AA classification had a good prognostic value in patients under 59 years of age. In older patients, no correlation was found between the classification and complete remission rate or survival time.
The authors report on their experience with the surgical treatment of syndactylia and flexor contracture in recessive dystrophic epidermolysis bullosa in a 7 1/2-year-old boy. After separation of the fused fingers by excision of the epidermal sac, all sclerotic tissue and affected skin, the defects are covered by split autograft skin taken from clinically non-affected areas. Morphologic and functional results are very satisfactory.
A comparative study was performed on platelets frozen with dimethyl-sulfoxide (D.M.S.O.), glycérol and dimethyl-acetamide (D.M.A.C.). The results of tests realized in vitro (platelet yields after thawing platelet aggregation, hypotonic stress, release of enzymes and ultrastructure), and in vivo (life span and recoveries after transfusion) have clearly shown that D.M.S.O. is the best cryoprotective agent. The results obtained with glycerol were less interesting and more clinical data are needed in order to study the hemostatic effectiveness of platelets preserved with this agent. Dimethyl-acetamide gave the worse results and in our opinion, must not be used for the cryopreservation of platelets.
Blood and bone marrow samples from 106 patients with Acute Myeloid Leukemia were reviewed independently by three observers according to the FAB proposals. Without a cytochemical stain, complete agreement occurred in 75% of cases. The major discrepancies were between M1 and M5 poorly differentiated (4 cases) and M2 and M4 (12 cases). These discrepancies disappeared with cytochemistry. Others non-concurrences were between M1 and M2 (4 cases) M2 and M6 (3 cases), M4 and M5 differentiated (2 cases), M2 and RAEB (1 case). To solve these latter discrepancies some points of the FAB classification require clarification.
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Ultrastructural cytochemical studies revealed peroxidase activity in five of 25 adult patients with apparent null lymphoblastic leukemia (ALL) in whom the peroxidase reaction studied with light microscopy was negative. None of these 5 patients responded to a chemotherapy regimen used for adult ALL. The importance of ultrastructural cytochemistry which allows the recognition of myeloblastic differentiation in undifferentiated blast cells is also demonstrated. The correct classification of such cases may be important for prognosis because they appear to be resistant to the chemotherapy used in treating ALL.
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