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Introduction: The management of pregnancy in young women with essential thrombocythemia is complex and may present a difficult problem. An adverse pregnancy outcome due to thrombosis or bleeding is a common complication. in addition, little is known about fertility in these women prior to the disease., Case Outline: We present the first case of a young woman with primary infertility and essential thrombocythemia who had uneventfully delivered a healthy boy in the fortieth week of pregnancy. Her platelet count was normalized during treatment with interferon-alfa. The patient failed to become pregnant in the natural way and after three attempts of programmed intercourse. She conceived only following intrauterine insemination. During pregnancy, the patient was carefully controlled by a hematologist and gynecologist., Conclusion: Natural course and prognosis of essential thrombocythemia is not adversely affected by pregnancy. In these women, the pregnancy should be planned only after normalization of platelet count. The interferon-alpha should be administered before the pregnancy to regulate and maintain the platelet count within the normal range. Intrauterine insemination with minimal hormonal stimulation due to the risk of thrombosis could be recommended as the safest treatment option of infertility in women with essential thrombocythemia.

Primary myelofibrosis is predominantly a disease of old age, poor prognosis and no curable treatment. Thrombocytosis was observed in only 12% of patients. To our knowledge, there is only one reported case of a young woman with primary myelofibrosis who had a term pregnancy [1]. We report on a 29-year-old woman with thrombocytosis and medical history of two miscarriages in the last 2 years, the iirst at 30 weeks of gestation and the second at 27 weeks. Multiple placental infarctions were observed. She was without symptoms but with moderate splenomegaly 4.5 cm below left costal margin). The platelet count was 651 x 10(9)/L, WBC 7.2 x 10(9)/L with normal differential formula, and haemoglobin level 12 g/dl. Bone marrow biopsy showed fibrotic phase of primary myelofibrosis, with hyperplasia of megacaryocytes, decreased numbers of erythroid and granulocytic cells, and increased amounts of reticulin fibres. Cyctogenetic examination of the bone marrow showed normal female caryotype. Increased numbers of progenitors CFU-Mk, CFU-GM and BFU-E were observed in peripheral blood, and decreased numbers in bone marrow cultures. As the patient wished to become pregnant, the treatment with interferon-a (Roferon A) was started at a dose of 3 MU s.c., three times per week. The platelet count rapidly decreased at a level of 260-370 x 10(9)/L. The pregnancy was diagnosed 5 months later. At the 24 week of pregnancy, platelet count raised to 690 x 10(9)/l and the dose of interferon-a was augmented, 3 MU every day, until delivery. Foetal growth and placental circulation were monitored by serial ultrasonography. At the end of 34 weeks of pregnancy, it was noted that placental flow became insufficient, and after foetal lung maturity was stimulated with dexamethasone, Cesarean section was performed. Male baby was born, weighting 2000 g, with respiratory distress syndrome. This complication was successfully treated, and the child is now one year old, with normal growth and development. The mother is still on therapy with interferon-a, 3 MU, three times a week, and the last blood count was as follows: haemoglobin 10.7 g/dl, WBC 6.1 x 10(9)/L and platelet comt 437 x 10(9)/L. In conclusion, according to the clinical results of interferon-d in thrombocytosis and experimental studies which showed the absence of placental transfer of interferon-d, this therapy could be recommended to women with primary myelofibrosis who wish to have a baby.

Introduction: The ability of interferon alpha and purine analogues to induce long-lasting remissions in the majority of patients with hairy cell leukaemia has revolutionized the treatment [16, 17, 24, 25], but also provoked dilemma and different opinions about the initial treatment or first line therapy. Splenotomy, the first standard treatment, increases peripheral blood counts but half of the patients require a later systemic therapy because of progressive cytopenia. On the other hand, it has been shown that splenotomy performed after achieving complete remission with interferon alpha or purine analogues contributed to spleen infiltration with hairy cells [37, 38]., Patients and Methods: In our study we analysed results of treatment of 44 patients with hairy cell leukaemia who were followed-up for 8 years. Patients were treated with three treatment modalities, mostly successively. Splenotomy++ was performed in 34 patients as a first line therapy (Group I); interferon alpha in 24 patients; in 10 patients as a first line therapy without splenotomy (Group II) and in 14 patients as a second line therapy after splenotomy (Group III). Deoxycoformycin was given to 5 patients as a third line therapy., Results: Our results showed that 20 patients (59 percent) treated only with splenotomy were in haematological remission (Group I). The analysis of prognostic variables demonstrated that at diagnosis patients from Group I, treated only with splenotomy, had at diagnosis higher levels of haemoglobin and thrombocyte counts and a lesser degree of bone marrow (b.m.) infiltration with hairy cells (HCs) than they were found in the other two groups of patients (median Hb = 11.3 g/dL, Plt = 133 x 10(9)/L, HCs in b.m. = 77%). Group II had median Hb = 8.6 g/dl, Plt = 72 x 10(9)/L, HCs in b.m. = 88%, and Group III had median Hb = 9.2 10(9)/L, Plt = 61 x 10(9)/L and HCs in b.m = 90%. Survival curves (Kaplan-Meier method) and Log Rank Statistics showed a significant difference in survival-time (Figure 4) among patients groups (p = 0.0427). Group I had median survival of 270 months; Group II 80 months; and Group III 140 months. Our results demonstrated that different prognostic risk groups existed among hairy cell leukaemia patients and could be identified on the basis of Hb level, Plt count and percent of infiltration of hairy cells into bone marrow. At diagnosis the low risk group of patients (Table 1) had levels of haemoglobin more than 11.3 g/dL; platelet counts more than 72 x 10(9)/L; percent of bone marrow infiltration with hairy cells less than 70%, and expression of lambda type of light chains on leukaemic cells. This group of patients was treated only with splenotomy without systemic therapy. In high risk group of patients the levels of haemoglobin were lower than 8.6 g/dL; platelet counts lower than 60 x 10(9)/L; percent of bone marrow infiltration with hairy cells more than 90% and expression of kappa type of light chains on leukaemic cells. The survival of this group of patients, in spite of initial treatment with systemic therapy with interferon alpha, was shorter. Results of treatment with interferon alpha (Group II and Group III) have demonstrated that the duration of remission in patients who were initially splenectomized and later treated with interferon alpha, was longer (median 31 months) than in patients who were treated with interferon alpha without splenotomy (median duration of remission = 13 months) (Table 3). Results of treatment with deoxycoformycin (Table 4) showed that 3/5 of patients achieved complete remission and 2/5 patients achieved partial remission. Four patients were initially splenectomised and att five patients were treated with interferon alpha before deoxycoformycin., Conclusion: On the basis of our results, splenotomy can be recommended as the first-line therapy for low risk patients with hairy cell leukaemia; interferon alpha should be the first-line therapy in high risk patients, and the second-line therapy in l