Case Presentation: We report the case of a 26-year-old gentleman of Malay ethnicity who presented to the medical department with a three-week history of abdominal distension associated with dyspepsia and epigastric pain. Physical examination revealed ascites. The complete blood count portrayed peripheral leucocytosis with eosinophilia of 8.84 × 109/L. Parasitic serology was negative. Paracentesis analysis showed exudative ascites with an absolute eosinophil count of 8 × 109/L. He was referred to the haematology department. He was noticed to have bilateral tonsillitis and pruritic skin rash at the legs. There were no palpable lymph nodes or organomegaly. A peripheral blood film showed 44% eosinophils with no excess blasts. Clonal eosinophilic fusion studies did not detect FIP1L1-PDGFRA mutation. JAK2 V617F and BCR-ABL1 mutations were undetected. Serum B12 and tryptase levels were normal. A whole-body computed tomography imaging showed bowel wall thickening at the duodenum, jejunum, ileum, rectosigmoid and splenic flexure. Sections of fragments taken from the endoscopy showed features of eosinophilic gastritis and colitis on histology. Bone marrow biopsy depicted marked eosinophilia. He was started on oral imatinib mesylate 200 mg daily and oral prednisolone 0.5 mg/kg daily which was tapered based on response. He achieved complete remission and is now asymptomatic.
Conclusion: The diagnosis of hypereosinophilic syndrome should be considered in a patient with unexplained ascites. Secondary sinister causes such as malignancy should always be excluded.
Methods: Ten patients with Glanzmann thrombasthenia aged 9 years (2009‒2018) were examined. Data on clinical characteristics, transfusion practices, and patient blood management were obtained from medical records. Patient blood management methods included parenteral iron, erythropoietin, hormonal pills, intrauterine progesterone contraceptive devices, tranexamic acid, and recombinant factor VIIa. Primary outcomes were hemoglobin levels and the proportion of patients who received blood transfusion. Secondary outcomes were morbidity and mortality.
Results: The median age at diagnosis was 8.2 years (range, 1‒15 yr). The female-to-male ratio was 9:1. Eight patients had type 2 disease (5‒20% of normal GPIIb/IIIa), and two patients had type 1 disease (normal GPIIb/IIIa <5%). All patients had iron deficiency. All female patients presented with significant menorrhagia. Other bleeding symptoms included epistaxis, spontaneous skin bruising, hemoptysis, gingival bleeding, knee hemarthrosis, and pelvic hematoma. No patient experienced life-threatening bleeding. Our patients had a mean hemoglobin level of 5.6 g/dL at diagnosis. All patients were optimized using non-transfusion methods as described above. Our patient had a current mean hemoglobin level of 11 g/dL. Approximately 70% (7/10) of patients did not experience receiving blood transfusions in the last 5 years. No patient experienced non-transfusion-related morbidities such as sepsis, thromboembolism, or cardiorespiratory events.
Conclusion: High cost, transfusion-related adverse events, and immunomodulation could be effectively prevented by avoiding unnecessary blood transfusions.