BACKGROUND: Myelofibrosis (MF) is a disease characterized by panmyelosis with intact maturation, progressive bone marrow fibrosis, and splenomegaly with extramedullary hematopoiesis. It is the most heterogeneous disease in the group of myeloproliferative neoplasms in terms of patient characteristics and natural history of the illness. Rationale and
OBJECTIVES: This study aims to describe the clinical, laboratory profile and survival of myelofibrosis patients seen in our institution and also to descriptively compare these characteristics of Filipino MF patients to Chinese and White MF patients.
RESEARCH DESIGN AND METHODOLOGY: An analysis of patients' data retrospectively collected through review of clinical records from 1994 to 2012 was done. All patients diagnosed with primary myelofibrosis (PMF) or post-polycythemia (post-PV) myelofibrosis or post-essential thrombocytosis (post-ET) myelofibrosis were included in this study. Survival was measured from first day of diagnosis to death from any cause or last known follow-up or direct communication to patient/relatives. Survival data were estimated using the Kaplan-Meier method. We compared the clinical characteristics and laboratory profile at diagnosis of these Filipino myelofibrosis patients to the Chinese as reported by Xu et. al., and White MF patients as reported by Cervantes et al.
RESULTS/SUMMARY: In our patients' data set, they seem to be younger with a median age of 57, less likely having constitutional symptoms but they have higher frequency of anemia, splenomegaly and leukocytosis. The heterogeneity of myelofibrosis' clinical and laboratory features were seen in this epidemiologic study on Filipino myelofibrosis patients in comparison with Chinese and White MF patients. The clinical course is a slowly progressive chronic disease with a median survival probability of 180 months.

Human ; Male ; Female ; Aged ; Middle Aged ; Adult ; Primary Myelofibrosis ; Splenomegaly ; Hematopoiesis, Extramedullary ; Leukocytosis ; Polycythemia ; Thrombocythemia, Essential ; Myeloproliferative Disorders ; Hematologic Diseases ; Anemia

OBJECTIVES: To describe the clinical course and treatment response of a case of thrombotic thrombocytopenic purpura (TTP) after a month of therapeutic plasma exchange (TPE) and immunosuppressive agents and to review related published literature regarding TTP and its response to TPE and immunosuppressive agents.
CASE AND DISCUSSION: A 64-year-old female presented with fever, bicytopenia, change in sensorium, and seizure of one day duration. Metabolic panel showed normal electrolytes with normal brain imaging. Hematologic work-up showed anemia, thrombocytopenia and leukocytosis with normal differential count. Reticulocyte was elevated. Peripheral smear showed significant schistocytes with nucleated red blood cells and marked thrombocytopenia. Thrombotic thrombocytopenic purpura was the initial consideration with the fulfilment of four out of the pentad of TTP, namely: microangiopathichaemolytic anemia, thrombocytopenia, fever and neurologic symptoms. Therapeutic plasma exchange was immediately initiated with daily platelet count and Lactate dehydrogenase (LDH) determination to assess response. However, despite daily plasma exchanges and continuous plasma infusion, there was inadequate response. In this light, immunosuppressive agents were started in the following order: high dose methylprednisolone, weekly rituximab and intravenous cyclophosphamide. On the 31st hospital day, after daily TPE and combined immunosuppression, she achieved complete response with a platelet count of 170 X 109/L to 250 X 109/L (baseline-14 X 109/L) and LDH 227U/L (baseline-1,191 U/L).
CONCLUSION: This study presented a challenging case of TTP which was successfully treated with the standard of care together with the available adjunctive treatment options.

Human ; Female ; Middle Aged ; Plasma Exchange ; Purpura, Thrombotic Thrombocytopenic ; Rituximab ; Thymidine 5'-triphosphate ; Lactate Dehydrogenase 1 ; Isoenzymes ; L-lactate Dehydrogenase ; Anemia