[The hypereosinophilic variant of Ph'-positive chronic myeloleukemia]. / Giperéozinofil'nyi variant Ph"-polozhitel'nogo khronicheskogo mieloleikoza.

AIM: To confirm clonal nature of idiopathic hypereosinophilic syndrome (IHES), its relevance to Ph'-positive chronic myeloid leukemia. MATERIALS AND METHODS: 3 cases of idiopathic hypereosinophilic syndrome are reported with morphologic analysis of bone marrow cells and cytogenetic examinations. In one patient the presence of Ph'-chromosome was confirmed at fluorescent in situ hybridization (FISH) and molecular-genetic analysis (bcr/abl). Samples of bone marrow, spleen and liver were examined pathohistologically. RESULTS: The presence of chromosome anomaly t(9;22), i.e. Ph'-chromosome, associated with chronic myeloid leukemia (CML) was identified in all the 3 cases. There was also myeloid hyperplasia in the bone marrow (with primarily mature, eosinophilic granulocytes), spleen and liver, depression of megakaryocyto- and erythropoiesis. 2 patients had similar clinical symptoms which was not typical for CML in chronic phase: fever, elevated ESR, clear-cut anemia and thrombocytopenia. In the absence of hyperleukocytosis, blood and bone marrow eosinophils remained high (42.5, 21.5, 42.5% and 21.4, 7.1, 6.5%, respectively) due to "mature" forms. The number of blasts in the bone marrow was maximum 2.4%. CONCLUSION: The literature and the obtained data suggest closeness of idiopathic hypereosinophilic syndrome and Ph'-positive CML within myeloproliferative diseases.

[Idiopathic and symptomatic hypereosinophilic syndromes (their comparative characteristics based on 14 cases)]. / Idiopaticheskii i simptomaticheskii giperéozinofil'nye sindromy (sravnitel'naia kharakteristika na osnove 14 nabliudenii).

Clinical, hematological, cytogenetic and pathohistological findings in 14 patients with high eosinophilia allowed the authors to distinguish 2 groups of patients: with symptomatic (secondary) and idiopathic hypereosinophilic syndromes (6 and 8 patients, respectively). The latter was characterized by hepato- and splenomegaly, specific cardiac lesion (thromboplastic endocarditis), non-infectious fever, anemia and thrombocytopenia, marked hypercellularity of the bone marrow with inhibition of erythro- and megakaryocytopoiesis. Ph'-chromosome occurred in 2 out of 8 cases. Biopsy and autopsy histology in all cases of idiopathic hypereosinophilic syndrome were typical for myeloproliferative diseases. In symptomatic hypereosinophilic syndrome the above features were not registered.

[A "primitive" variant of the blast crisis in chronic myeloleukemia]. / "Primitivnyi" variant blastnogo kriza khronicheskogo mieloleikoza.

While immunotyping blast cells from 45 patients with CML blast crisis, we detected 5 cases with immunologically primitive blast cells. The immunological phenotype of these cells corresponded to that of primitive stem cells which are characterized by expression of CD34 and HLA-DR antigens in the absence of other immunological markers. We suggest that blast cells from these patients may undergo differentiation similar to that of primitive stem cells that implies the existence of a new immunological variant of CML blast crisis, a primitive variant. Morphologically, blast cells in 3 cases could be classified as myeloid, in 2 cases precise identification was impossible. Cytochemically, this type of cells can be defined as mixed. The patients with CD34+ phenotype do not differ clinically or hematologically from those with CML blast crisis. Blast cells with membrane marker CD34 are likely to arise in any CML phase either as a component of overall leukemic population or predominant, single subclone.

The diagnostic significance of serum ferritin indices in patients with malignant and reactive histiocytosis.

Iron metabolism was studied in 10 patients with malignant histiocytosis (MH), in 16 patients with histiocytosis-X (Langerhans cell histiocytosis) and in 34 patients with reactive proliferation of the mononuclear phagocytes (MPS). Eight MH patients had a considerably increased level of serum ferritin (SF). The average level of SF was 6070 +/- 957 mg/l for MH patients, which is significantly greater than the SF level for HX and RH patients. The study of the serum ferritin profiles suggests that the main pathogenetic mechanism of hyperferritinaemia in MH is the synthesis and secretion of ferritin by neoplastic mononuclear phagocytes. From this, hyperferritinaemia in MH may be considered as pathognomic of this disease and used as an additional diagnostic criterion. In addition, SF is the most informative parameter for the clinical practice among the indices of the functional state of MPS.

[The treatment of histiocytosis X (Langerhans-cell histiocytosis) with alpha-interferon preparations]. / Lechenie gistiotsitoza-X (gistiotsitoz iz kletok Langergansa) preparatami al'fa-interferona.

Langerhans cell histiocytosis (LCH) and histiocytosis class II (without Langerhans cells) were treated in 10 and 2 patients, respectively, with alpha-interferon preparations: recombinant alpha 2-interferon and two combined preparations containing natural alpha-interferon and immune response phase 1 cytokines. The patients' age ranged from 18 to 61 years, all of them had multiple lesions. Six of them exhibited dysfunctions of the affected organs. As shown by the results of 16-52-month follow-up, LCH patients' disease persisted in chronic active stage without progression (8 cases) or reached a complete remission (1 case), one patient died. In histiocytosis class II alpha-interferon administration was uneffective.

[Generalized mastocytosis--hemoblastosis of mast cells]. / Generalizovannyi mastotsitoz--gemoblastoz iz tuchnykh kletok.

A review of literature has been presented including current data on bone marrow genesis functional role of mast cells in the human body, as well as characteristics of the main clinical variants of mastocyte tumor proliferations (solitary mastocytoma, urticaria pigmentosa, generalized mastocytosis). Clinicomorphological picture and positive results of the treatment with the Soviet cytostatic spirobromine of two patients suffering from generalized mastocytosis are described.

[Histiocytosis X--histiocytosis from Langerhans' cells]. / Gistiotsitoz Kh--gistiotsitoz iz kletok langergansa.

A review of literature has been presented involving clinicomorphological characteristics of certain variants of histiocytosis X (HX) and current conception considering this disease as an immunopathologic process that is underlain by reactive proliferation of a specific subtype of antigen-presenting macrophages--Langerhans' cells. Clinico-morphological picture has been described and the results of successful treatment with roferon (human gene-engineering alpha 2-interferon) in 3 adult patients with HX have been presented. It is stressed that the actual effectiveness of the treatment with roferon could not be critically evaluated due to a high frequency of spontaneous remissions of this disease.

[Heart involvement in multiple myeloma]. / Porazhenie serdtsa pri mnozhestvennoi mielome.

The results of the myocardial functional capacity study in 54 patients with multiple myeloma were compared with the data of clinical and anatomopathologic investigations. No specific clinico-functional signs of cardiac affection in multiple myeloma, having differential diagnostic importance, were revealed. Specific affection of the heart was detected in 30% of cases during anatomopathologic investigations.

[Effectiveness of plasmapheresis on the PF-0.5 blood separator in patients with paraproteinemic hemoblastoses]. / Effektivnost' plazmafereza na fraktsionatore PF-0,5 u bol'nykh paraproteinemicheskimi gemoblastozami.

Plasmapheresis on a Soviet-made PF-0.5 blood cell separator was performed in 20 PPH patients (altogether 33 sessions of plasmapheresis). 1600 to 3300 ml of plasma were removed during one session. Rheopolyglucin was mainly used as plasma-substituting liquid. The procedure was well tolerated by all the patients. After plasmapheresis biochemical indices were within normal. In multiple myeloma paraprotein was decreased by 42%. Waldenström's macroglobulinemia by 47%. Plasma viscosity was decreased 1.2- and 2-fold, respectively. The investigations showed a high efficacy and reliability of plasmapheresis with the help of the Soviet-made PF-0.5.