A 63 year old woman presents with melena for 3 weeks, and fatigue and malaise for 2 weeks. No other symptoms were present. Her medical, surgical and drug histories are unremarkable, and there is no family history of bleeding disorders or gastrointestinal malignancies. She does not smoke, and only drinks socially.
Hb: 7.5 g/dL (12.0-15.0) RBC: 2.52 x 10^6/mm3 (4.0-4.9x10^6) Reticulocytes: 2.5% (0.5-1.5) WBC: 5,900/mm3 (4,500-10,500) Plt: 144x10^3/mm3 (150-400x10^3) The peripheral blood smear shows immature myeloid cells, tear-drop shaped cells, nucleated red cells, and giant platelets.
AST: 17 IU/L (8-48) ALT: 17 IU/L (7-55) ALP: 771 IU/L (45-115) LDH: 525 IUL (122-222) GGT: 86 IU/L (9-48) Total protein: 7.0 g/dL (6.3-7.9) Albumin: 3.9 g/dL (3.5-5.0) Total Bilirubin: 2.63 mg/dL (0.3-1.9) Direct Bilirubin: 0.76 mg/dL (0-0.3) Hepatitis B and C virus markers: negative. The ultrasound scan of the abdomen reveals massive splenomegaly and a dilated splenic vein. The liver appears normal, with no features suggestive of cirrhosis.
Despite multiple attempts, bone marrow aspiration results in a dry tap. The trephine biopsy is normocellular (40% marrow cellularity), with a patchy distribution of myeloid and erythroid cells, and normal numbers of non-clustered megakaryocytes, with some having hypolobated nuclei and bare megakaryocyte nuclei. Scattered interstitial lymphocytes and focal nonparatrabecular aggregates of mature lymphocytes were present. No intrasinusoidal hematopoiesis is seen. Reticulin is reported to be grade 3.
Endoscopy reveals grade III esophagogastric varices at the 3 o'clock and 9 o'clock positions. A white thrombus is noticed on the middle of the 9 o'clock varix.