Belcheva, A.

Dineva, V.

Kudeva, K.

Mishkova, R.

Nikolova, G.

Stoynova, L.

Zahariev, A.

Zhelyazkov, D.

English

Varna Medical University Press: Journals

C L I N I C O — M O R P H O L O G I C A L C H A R A C T E R I S T I Q U E A N D T R E A T M E N T O F M Y E L O D Y S P L A S T I C S Y N D R O M E S R. Mishkova, A. Zahariev, A. Belcheva, D. Zhelyazkov, K. Kudeva, G. Nikolova, V. Dineva L . Stoynova Key-words: myelodysplastic syndrome — clinics — morphology — treatment — prognosis The rising interest in the problem of myebd/spbst i : s y a l n i t e s ( 4 D 3 ) is recently determined by the new data about the peculiarities of leukemic trans­formation of stem haematopoietic cells. MDS classification is still specified and problems of the therapy are discussed (4, 7, 8, 13). ( In the present communication we demonstrate thi e l i n i o m o r ) п м э ] 1 :al peculiarities and our experience with the treatment of MDS. Material and methods During a 10-year period, in the Hematologic Clinic of the Higher Institute of Medicine, Varna a total of 18 patients (12 males and 6 females) aged between 22 and 82 years with a mean age of 62.5 years were investigated. A MDS was diagnosed on the basis of clinieo-morphDlogieal and eytochemical data as well. The following parameters were followed-up in these patients: hemoglobin, leu­kocyte count with differential blood picture, thrombocyte count, bone-marrow puncture, and in single patients only — trepano-biopsy, reaction for glycogen and peroxidase in blast elements, blood histamine level determined by spec-trophotoflurimetry. Diseases and states in which megaloblast haemopoiesis can be observed e. g. B 1 2 or folic acid deficit, liver diseases, chronic ethylism and renal failure were excluded in all the cases. Results and discussion MDS kind was specified according to F A B classification criteria (4). 1. Refractory a n a e m i a — Ц patients. 2. Refractory anaemia with blast «excess» in the bone marrow (between 0,06 and 0.20) — 2 patients. 3. Chronic myelomonocytie leukemia — 2 patients. 4. Refractory anaemia with «excess» of blast cells in transformation (more than 0,20 — 0.30 in the bone marrow and over 0.05 in the peripheral blood) — 3 patients. Hemoglobin values were between 60 and 80 g/1 in all the patients. T h e y worebelew 40 g/1 in two patients only. Mean erythrocyte volume was 117.8^ 4-2 3 and thus over the normal value.Morphologically, mecrocvtosis and megajo-Clinico-morphofogkal characteristique and. 69 ytosis was fount out. There were ovalocytes and stomatocytes in two patients ut basophilically punctated erythrocytes in other two ones. Erythroblasts J peripheral blood were observed in four pa'tients (between 0.02 and 0.38). Granulocyte ount v fied in single patients It was below 3/0 x 10*/1 in? cur cas'^s, it wis over 10 x 109/1 in three ones a id within norma!! limits (at'th'e average 5.4 0.7 < 10 9 i ) in the rest patients. Hvposegmentation as an anomaly in granulocyte morpholog/ wis established in one patient only. A transformation with appearance of younger cells (up to myeloblasts) in the peripheral! blood (betwe n0.20 and 0,30) was established in three patients with refractor/ artae-ia in the coarse os the disease. Blood histamine level was repeatedly followed-p within an interval of 2 — 6 months in order to exclude a myeloproliferative process in these patients. This index was far below the limiting value concerning •hronic myelogenic leukemia of 1.0Эц g/ml, namely between 0.044 and 0.150 g/ml. The dynamic folbw-uo revealed lymphocytosis over 0.50 accompanied by a considerable lymphocytic infiltration in the bone marrow in 6 patients. In four of them, this abnormality preceded the clinical manifestation of an acute non-lymphoma (in the rest two). Monocyte number was 00.40 and 0.30, respecti­ve ly / in two myelomonoeytic leukemia patients. Thrombocyte count was sub­normal in all the patients (107.6 ± 1 0 . 2 g/1). Some features of the myelogram are stressed, indeed. There is an erythro­blastic hyperplasia with signs of megaloblast haemopoiesis refractory to vita­min B 1 2 or folic acid treatment in all the patients. Serum iron levels are between 30 and 54[i mol/1 in 11 patients and in normal limits in the rest. The problem of the treatment of MDS patients is rather controversial. Some authors report a positive effect of alexan application at low dosages (3, 5, 6, 10, 12, 14, 15). Other investig ators avoid the incorporating of cytostatic means. An attempt was made to treat three refractory anaemia patients by usin? of ale­xan at a dosis of 20 mg/m2 daily for 21 days. However, no significant change of the clinical course of the disease and of haematological parameters in compa­rison with the treatment with corticosteroids and blood transfusions as in the rest patients could be observed. It has to be noted that this failure cannot re­ject this schedule of MDS treatment at al l . On the contrary, the problem of the choice of MDS variants appropriate to such a treatment requires to be solv­ed as soon as possible. The evolution of the disease varies in single patients — from some months up to 10 years and more (in two cases). These patients need intensive care and they have been hospitalized many times (at the average 7 — 8 times yearly) due to the necessity for correction of the anaemic syndrome mainly. In conclusion we point out that concerning their clinico-morphological manifestation MDSs are many-sided and thus pose diagnostic and therapeutic problems. Their possibility to transform into aeute leukemia requires a dynamic follow-up of the patients as well as the introduction of new diagnostic methods (colony-forming capacity of early granulocyte and erythrocyte precursors and active search for chromosomal abberations) ( 1 , 2, 8, 9, 11) into the clinical prac­tice. 70 R. Mishkova, A. Zahariev, A. Belcheva, D, Zhelyazkov, K. Kudeva, G. Nikolova,. . . R E F E R E N C E S I . Г р и г о р ь е в а , Г. M . t С. С. С о б о л е в а , Н. В. Ц в е т а е в а . Гема-тол. и трансфузиол., 1985, № 9, 10—12. — 2. Л о р и я, С. С , Л . И. Д е р б е н е в а , Д . 3. Т о б а г а р и, 3. Г- К а д а г и д з е, Ю. И. Т о к а р е в. Гематол. и транс­фузиол., 1985, Ко 2, 38—41. — 3. В а с с а г a n i , М., G . F a s о 1 a, R . F а п i п. et a l . Haematologica, 70, 1985, 160—165. — 4. B e n n e t t , J . M. , D. C a t o v s k y , M. T . D a n i e l , et a l . Brit. J. Haematol., 51 , 1982, 189—199. — 5. С a s t a i g n e, S. , M. T . D a n i e l , H . T i l l yf P . И е r a i t, L . D e g o s . Blood, 62, 1983, No 1, 85—86. -6. D e s f o r g e s , J . F . New Engl. J. Med., 309, 1983, 1637 — 1639. — 7. F о u с a r, K„ R . M . L a g d о n, J . О. A r m i t a g е, B . D. O l s о n, T . J . C a r r o l l . Cancer, 56, 1985, 532—534.— 8. G e a r y , C . G . Brit. J. Haematol., 55, 1983, No 1, 1 —0. — 9. G г е е n b е r g, P . , G . В a g b y. Brit. J. Haematol., 53, 1983, 532—534. — 10. L a r, s o n , R . A . Ann. Intern. Med., 103, 1985, 136—138. — 11. M a v, S. J . , S. A. S m i t h -A. J a c o b s , A. W i l l i a m s , R . B a i l y-W о о /. Brit. J. Haematol., 59, 1985, 311—319. — 12. M i с h е 1 s, S. D., R . W . M e k e n n a , D. C . A r t h u r , R . D. B r u n n i n g . Blood, 65, 1985, 1364—1372.— 13. M u f t i , G . J . , J . R . S t e v e n s , D. G . O s c i e r, et a l . Brit. J. Haematol., 59, 1985, 425—433. — 14. R о b е r t s, J . D. , W. В . Е r s h i e r , В . H . T i d 1 e, J . A. S t e w a r t . Cancer, 56, 1985, 1001—1005. — 15. W i n t е r, J . N . . D. V a r r i a k o j i s , E . R . C a v n o r , R . A . 1. a r s o n , К. B . M i l l e r . Cancer, 56, 1985, 443-^449. КЛИНИКО-МОРФОЛОГИЧЕСКАЯ ХАРАКТЕРИСТИКА И ЛЕЧЕНИЕ МИЕЛОДИСПЛАСТИЧЕСКИХ СИНДРОМОВ Р. Миткова, А. Захариев, А. Белчева, Д. Желязков, Д\ Кудева, Г. Николова, В. Дннева, Л. Стойнова Р Е З Ю М Е ' — Ь ) . . . У восемнадцати больных с миелодиспластическимн синдрома ми установлена морфо­логическая и цитохимическая характеристика периферической крови и костного мозга, а также и особенности клинического протекания. Для дифференциальной диагностики миело-диспластических синдромов и миелопролиферативных заболеваний использован критерии уровня гистамина в крови. У трех больных проведено лечение небольшими дозами алсксана. В работе обсуждается терапевтический эффект препарата. • 

CLINICO-MORPHOLOGICAL CHARACTERISTIQUE AND TREATMENT OF MYELODYSPLASTIC SYNDROMES

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CLINICO-MORPHOLOGICAL CHARACTERISTIQUE AND TREATMENT OF MYELODYSPLASTIC SYNDROMES

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