MIXED IMMUNODEFICIENCY, ATYPICAL MYCOBACTERIA AND MYELOFIBROSIS

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Background and Aims. Myelofibrosis can be idiopathic (a chronic myeloproliferative syndrome) or secondar y to many kinds of insults, as a reaction to malignancy, infections, endocrinopathies, auto-immune diseases and granulomatous disease. Pancytopenia and hepato- splenomegaly are frequent, and only reversible when the secondary injury can be treated. The authors present a case of myelofibrosis diag- nosed in an 11 months old boy; later it was discovered to be secondary to atypical mycobacteria and quadruple antibacillar therapy for one year reverted the clinical status. Methods/Clinical Case: A caucasian male infant 4 months old presents with anaemia and pneumonia with pleural effu- sion. Three months later , he has a peri-anal abscess and presents with hepato-splenomegaly and pancytopenia. The analysis revealed Hb 8,0g/dL (with presence of erythroblasts, frank anisopoikylocytosis, and dacriocytes), leukocytes 1500/mm Backgrounds. Basic diagnosis of essential thrombocythemia is proved by estimation of elevated platelets count and corresponding findings of activated megakaryopoiesis in bone marrow with contemporar y exclud- ing their reactive changes. The therapy is mainly focused on correction of high platelets count in the blood. The treatment is different in young and elderly patiens, in cardiovascular or thrombotic risk and non-risk patiens. However, the treatment influences not only the count, but also the function of platelets and so can lead to influencing of clinical symp- toms. The function of platelets is not currently investigated before drug adminstration and in the course of the treatment. Aim of the study was to evaluate clinical and laboratory importance of platelet functional char- acteristics in essential thrombocythemia. Methods. 30 patients have been included in our observation and we performed (be side the basic labora- tory tests) platelets aggregation according to Born, PFA-100 examina- tion and flow-cytometric estimation of CD36, CD42a, CD61, CD62, CD63 markers. The laboratory testing was done before and six months after the treatment. (The platelet aggregation was tested using ADP in two concentrations, colagen and cationic propylgalat as inductors, and three parameters - percentil of aggregation, slope and desaggregation remark - were evalutated). Results. the decreasing of aggregation response (in 16 cases after all inductors used) was not accompaned by statisticaly significant changes in other examinations of platelet function tests. Moreover, there were not observed statisticaly significant changes in repeated examinations after six month of the treatment. There was even no correlation between functional examination of the platelets and clin- ical symptoms of the disease. Conclusion: Functional disorder of the platelets seems to be the part of clinical findings of the disease, but does not correspond with biological activity of the disease or with its clinical symptoms and/or with the answer to the therapy. Although, the treat- ment (especially with acid acetylosalicylic-ASA) can widely modify platelet function, it was not even observed to be significantly different in our ASA-treated vs. ASA-nontreated patients. 1472

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