[Human leukemias studied with monoclonal antibodies to erythroid cells]. / Izuchenie leikozov cheloveka s pomoshch'iu monoklonal'nykh antitel k éritroidnym kletkam.

Activity of monoclonal antibodies HAE3 and HAE9 specific for human erythroid cells to different leukemic cells is described. These monoclonals do not react with nonerythroid leukemic cells. HAE3 and HAE9 reactivities are similar to those of polyclonal monospecific antibodies against an antigen of erythroblasts--a surface antigenic marker of nucleated red cells and reticulocytes. Monoclonal antibodies HAE3 and HAE9 are proposed to be used in diagnosis of leukemias.

[Characteristics of two new mutant forms of erythrocyte glucose-6-phosphate dehydrogenase: "Kirovograd" G6PD and "Zhitomir" G6PD]. / Kharakteristika dvukh novykh mutantnykh form gliukozo-6-fosfatdegidrogenazy éritrotsitov: G6PD "Kirovograf" i G6PD "Zhitomir"

The paper comprises the description of properties of three mutant forms of glucoso-6-phosphate dehydrogenase characterized according the WHO program. Preparations of the enzymes were isolated from erythrocytes of patients with G6PD deficiency from three unrelated to one another Ashkenasi families coming from the Ukraine and from Byelorussia. Two new variants of G6PD hitherto never described in the literature were discovered. These variants were designated as "Kirovograd" and "Zhitomir" after the towns the probands came from. The properties of purified enzymes revealed by the methods of the WHO program were as follows: the variant "Kirovograd" has a normal electrophoretic mobility in tris and TEB buffers and 98% of the normal in phosphate buffer. KM for G6P is 6,54; KM for NADP--2,19. It is characterized by a reduced thermostability and by an acute peak of activity at pH 8,5. The variant "Zhitomir" has 90-98% of the normal electrophoretic mobility in TEB buffer and 78-84% in phosphate buffer. KM for G6P is 5,4-8,3. KM for NADP is 1,4-3,1; with deoxyG6P is 50% and with deaminoNADP is 35%. It is also characterized with a reduced thermostability, while the curve of the dependence of its activity on pH has two peaks. Both variants are perfectly inactive with erythrocytes and thus should be assigned to the second group of the mutants variants of G6PD. The comparison of these variants to other variants encountered in the same national group revealed that they resemble certain quantitative variations.

[Study of the molecular causes of thalassemia. II. Deletion of alpha-globin genes in hemoglobinopathy H]. / Izuchenie molekuliarnykh prichin talassemii. Soobshchenie II. Deletsii alpha-globinovykh genov pri gemoglobinopatii H.

Genetic and molecular studies on one case of alpha-thalassemia, found in Moscow region, have demonstrated that the cause of the disease is the deletion of 3 of 4 alpha-globin genes, presenting in the human diploid genome. The level of expression of the remained alpha-globin gene is much lower than that observed in the patients from other ethnic groups. One can suggest that in this case the deletion is spread on the regulatory zones of this gene.

[New variant of glucose-6-phosphate dehydrogenase (G-6-PD "Kaluga") from erythrocytes of a patient with chronic nonspherocytic hemolytic anemia]. / Opisanie novogo varianta gliukozo-6-fosfatdegidrogenazy eritrotsitov (G-6-FD "Kaluga"), soprovozhdaiushchegosia khronicheskoi nesferotsitarnoi gemoliticheskoi anemiei

Properties of a new form of glucose-6-phosphate dehydrogenase (G6PD) from erythrocytes, called "Kaluga", are described. The enzyme was isolated from erythrocytes of a patient with chronic non-spherocytic hemolytic anemia; in the blood cells 20% of the G6PD activity was maintained as compared with normal state. The partially purified enzyme was shown to be unstable in electrophoresis, it possessed a biphase type of pH-optima; Km value for NADP was decreased for glucose-6-phosphate Km value was normal. The thermostability of G6PD was normal at 46 within 1 hr.

[Effectiveness of the treatment of severe aplastic anemia (splenectomy and immunosuppression)]. / Otsenka éffektivnosti lecheniia tiazheloi aplasticheskoi anemii (splenéktomiia i immunodepressiia).

The authors appraise the efficacy of the treatment of severe aplastic anemia by splenectomy, antilymphocyte globulin and cyclophosphamide. A total of 120 patients were placed under observation. Of these, 103 patients had a severe disease pattern, one hundred and 112 patients with aplastic anemia including 96 with a severe pattern were subjected to splenectomy. Of the 112 splenectomized patients, 78 (69.6%) are alive. Of the 96 patients with severe aplasia, 60 patients (62.5%) are alive. Of 43 deceased patients, 7 had been admitted to the hospital 2-12 days before death and had not been subjected to splenectomy. Three patients died shortly after splenectomy, 8 patients died at the second month after operation. During the first 6 months after operation 20% of the operated patients died, whereupon 10 more per cent of the patients died for 5 months. The patients who survived 10 months may happen to develop a complete or partial remission. Complete remission was attained in 18.8% of the patients, almost complete recovery in 17.7% of the operated, considerable improvement of the estimates was attained in 17.7% of the patients. Antilymphocyte globulin was administered to 23 splenectomized patients. The remission was attained in 6 patients, and 8 more patients improved. If the patients did not respond to the treatment with antilymphocyte globulin, or if they developed relapses, the authors administered cyclophosphamide. Of the 15 patients, 9 responded to the treatment (5 patients improved, remission was attained in 4 patients, 3 of whom are in a state of remission until now). On the 15 patients treated with cyclophosphamide 12 patients have been alive for not less that 2 years.

[Antithrombocyte antibodies in the serum and on the surface of the thrombocytes in patients with chronic lymphoproliferative diseases]. / Issledovanie antitrombotsitarnykh antitel v syvorotke i na poverkhnosti trombotsitov bol'nykh khronicheskimi limfoproliferativnymi zabolevaniiami.

Antiplatelet antibodies were studied in patients with chronic lymphatic leukemia (CLL) using ELISA for detection of serum antibodies and RIA for determination of antibodies associated with platelet surface. Serum antibodies were identified in 1 out of 6 CLL patients with the platelet count 100,000-200,000 per microliter and in 7 out of 54 CLL patients with the platelet count lower than 100,000. Platelet-associated antibodies were not detected in patients with the normal platelet count, but were revealed in 14 out of 25 patients with the platelet count 100,000-200,000 per microliter and in 21 of 27 patients with the platelet count lower than 100,000 per microliter. The presence of a group of thrombocytopenic patients lacking serum or platelet-associated antibodies suggested that the development of thrombocytopenia in CLL could be mediated not only by antibodies but also by other pathogenic mechanisms, i.e. by the depression of megakaryocytes in bone marrow. Increase of the platelet count after steroid and cytostatic treatment in CLL patients correlated with decrease of antiplatelet antibodies. In splenectomized CLL patients, the increase of the platelet count and disappearance of hemorrhage occurred in patients with the low level as well as with the high level of antibodies after the operation.

[Erythromyelosis in the outcome of partial red cell aplasia]. / Eritromieloz v iskhode partsial'noi krasnokletochnoi aplazii.

The authors describe two women with a history of partial red-cell aplasia of the bone marrow without any remissions for 2 and 3 years, followed by the development of erythromyelosis. The latter lasted from 1 to 2 years, the total illness duration being 4 to 5 years. All the attempts to achieve remissions with the use of splenectomy, mono- and polychemotherapy with a purpose of immunosuppression ended in failure. Partial red-cell aplasia is viewed as preleukemia.

[Autoimmune thrombocytopenia and diffuse-toxic goiter]. / Autoimmunnaia trombotsitopeniia i diffuzno-toksicheskii zob.

The authors describe 10 patients with associated diffuse toxic goiter and thrombocytopenic purpura and a female patient with associated goiter and a three-shoot autoimmune peripheral cytopenia. In 8 patients thyrotoxicosis preceded the appearance of thrombocytopenia, in 3 patients, both the conditions were diagnosed at a time. In 4 patients, the measurements were taken of the IgG content on the surface of platelets according to Dixon et al. In 3 patients, the IgG content turned out to be appreciably elevated, in one patient, the content of IgG was within normal, however the latter patient was examined after prednisolone intake that had given rise to an increase in the platelet count. Two patients who received radioactive iodine and three patients treated by thyrostatic drugs were later on subjected to splenectomy. In 4 patients, thrombocytopenic purpura remitted after subtotal strumectomy. One female patient was subjected to sectoral resection of the thyroid. Two years after surgery the patient, who was in an euthyroid state, developed thrombocytopenia which required splenectomy. It is possible that in the latter case there was no direct relation between the two diseases. The relationship between the two autoimmune diseases, diffuse toxic goiter and thrombocytopenic purpura, remained unclear in other 10 cases. The relationship between the diseases under consideration and approaches to specifying the character of such a relationship are under discussion.

[Aplastic anemia after acute viral hepatitis]. / Aplasticheskaia anemiia posle ostrogo virusnogo gepatita.

The authors describe 4 patients with grave aplastic anemia that developed after acute virus hepatitis. In two cases aplasia occurred at the icteric period of hepatitis, in one during convalescence, and in one 5 months after the recovery from hepatitis. The counter electrophoresis technique failed to reveal the Australian antigen in all the 4 cases. Ninety per cent of patients out of over 200 reported cases of aplastic anemia that developed after acute virus hepatitis died. Of the 4 cases followed up by the authors, 3 patients died. One of the female patients was subjected to splenectomy 3 weeks after the occurrence of grave aplasia with fatty bone marrow with a purpose of immunodepression. Splenectomy entailed a considerable decrease in hemorrhagic diathesis. Later on the patient was treated with caprine antilymphocytic globulin. At present the patient is in a state of remission. The problems of the pathogenesis of aplastic anemia following acute virus hepatitis and potentialities of the disease treatment are under discussion.

[Neoplastic form of chronic erythromyelosis (erythrosarcoma) with eosinophilia and cardiac lesions]. / Opukholevaia forma khronicheskogo éritromieloza (éritrosarkoma), protekaiushchego s eozinofiliei i porazheniem serdtsa.

The paper is concerned with a case history of a 24-year old man suffering from chronic erythromyelosis with erythrokaryocytic metaplasia of the peripheral lymph nodes, bone marrow, spleen, liver, heart and lungs. The hypereosinophilic syndrome and endocardial fibrosis caused diagnostic difficulties. A short-term effect after injection of the plasma from a patient with erythrocytic aplasia containing antierythroblastic antibodies was obtained, tumor tissue mass reduced. Later on courses of CAMP-therapy were initiated. The progression of disease resulted in the patient's death in 2 years.

[Generalized immune-complex vasculitis combined with pulmonary hemosiderosis and dilated cardiomyopathy]. / Generalizovannyi immunokompleksnyi vaskulit v sochetanii s legochnym gemosiderozom i dilatatsionnoi kardiomiopatiei.

The paper is concerned with observations over 3 patients in whom unusual vasculitis lay at the basis of the clinicopathological manifestations. All the patients were men of the young age. The disease debut was marked by fever, weakness, dyspnea, palpitation, cough, hemoptysis, the articulation syndrome. In two cases, there was hemorrhagic rash on the leg skin. All the patients manifested liver and spleen enlargement, two patients had lymphoadenopathy. The leading clinical symptoms included dilated cardiomyopathy, complete blockade of the inferior peduncle of His bundle and reduction of myocardial contractility. Anemia belonged to iron deficient one. The clinical examples provided indicate that immunocomplex vasculitis with evident lesions of the lungs and myocardium, not going into criteria for the known diseases, is not likely to be a casuistic rarity. Those syndromes may be associated with more or less pronounced hemosiderosis of the lungs (and, probably, of the lymph nodes, spleen and liver), with transitory or steady derangements of myocardial conduction, which attests to diffuse lesions of the myocardium possibly with both immune complexes and hemosiderin. The pathology requires further studies.