Danazol therapy combined with intermittent application of chemotherapy induces lasting remission in myeloproliferative disorder (MPD): an alternative for the elderly with advanced MPD.

There is no good alternative therapy available for elderly patients with advanced myeloproliferative disorders (MPD) who failed on conventional therapies and are not candidates for bone marrow transplant. We report here an effective therapy that induced exceptionally long-lasting remissions and improved quality of life. Eighteen elderly patients (mean age: 70·6 years) (16 myelofibrosis and 2 thrombocythemia) who had failed on conventional therapies were treated. Danazol was administered daily at 200-800 mg throughout the study. Chemotherapy was applied intermittently as needed to reduce spleen size and blood counts. Busulfan (2-4 mg/day) was used most often and 6-mercaptopurine (6-MP) (50-100 mg/day) and/or cytarabine (100-200 mg/m(2)) if the white blood cell (WBC) count rose rapidly. When MPD stabilized, chemotherapy was discontinued and dosage of danazol was reduced. Therapy was well tolerated. Overall, 61% of patients responded with unexpectedly long-lasting remissions and improved quality of life. Three (17%) had excellent (E) response, defined by normalization of blood counts and non-palpable spleen, while eight (44%) had good (G) response, defined by rise of Hct by ≥7% and ≥50% reduction of spleen. Mean duration of remission was 45 months (10-78 months) in E responders and 11 months in G responders (2-22 months). This regimen offers a safe and effective alternative for advanced MPD in the elderly.

Interleukin-11 for treatment of hepatitis C-associated ITP.

BACKGROUND: Immune thrombocytopenic purpura (ITP) is frequently associated with chronic hepatitis C (HpC-ITP). METHODS: Recombinant interleukin-11 (rIL-11), which has both thrombopoietic and anti-inflammatory properties, was evaluated in 12 patients with HpC-ITP in this pilot study. Group 1 (7 patients) was treated at high dose (50 microg/kg daily) while group 2 (5 patients) at low dose (15-35 microg/kg three/week). RESULTS: In group 1, mean platelet counts rose from initial 54 x 10(9)/l to 103 x 10(9)/l (p = 0.02) and in group 2, from an initial 51 x 10(9)/l to 74 x 10(9)/l (p = 0.04). Antiplatelet antibody (aPlt-Ab) decreased in group 1. LFT improved in both groups. The mean HCV-RNA decreased in group 1 (p = 0.04), not in group 2. Side effects were common and troublesome, but were minimized with individualized dosing. One patient achieved good remission of both ITP and HpC lasting >2 years with low-dose maintenance. CONCLUSION: When used based on individual tolerance, rIL-11 appears useful in HpC-ITP.

Interstitial lung disease (ILD) and severe ITP.

INTRODUCTION: Platelets play an important role in inflammatory and immune responses. We report interstitial lung disease (ILD) developing during the acute phase of severe thrombocytopenia in 3 patients with severe refractory ITP. METHODS AND RESULTS: We identified 3 cases with severe ITP who developed ILD in the course of refractory chronic ITP. The thrombocytopenia was severe in all cases. ILD was an incidental finding at the presentation and often misdiagnosed as lung infections. ILD was documented by lung biopsy in cases 1 and 2, supplemented by serial chest X-rays and/or CAT scan. As the ITP improved, ILD regressed in case 1, persisted in case 2, and progressed to advanced pulmonary fibrosis in case 3. CONCLUSION: We report an association of ILD with severe refractory ITP. ILD was detected in acute phase of platelet destruction, suggesting that platelet destruction may have triggered inflammation in the lung, leading to ILD.