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Primary Myelofibrosis


Step 1: View clinicals

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.

Step 2: Order all relevant investigations

Complete Blood Count + Peripheral Smear

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.

Liver Profile + Ultrasound Abdomen

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.

Bone Marrow Aspiration + Biopsy

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.

Upper GI Endoscopy

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.

Step 3: Select appropriate management

Stem Cell Transplantation
Blood Transfusion
Endoscopic Variceal Ligation

Score: ★★☆