Myelofibrosis

Failing

Step 1: View clinicals

A 70-year-old man is referred for further evaluation by his primary care practitioner, following the detection of anemia in a complete blood count. His only complaints are worsening fatigability and exertional dyspnea for six months. His medical history is positive for essential hypertension for five years and type 2 diabetes mellitus for one year, both of which are uncomplicated, and well-controlled via diet and exercise alone. He is not on any medications currently. His surgical and family histories are unremarkable. His diet is nutritionally balanced, containing meat, fish, and vegetables. He only drinks socially and aside from very short-term experimentation in his youth, has not smoked. There is no history of allergies.


Step 2: Order all relevant investigations

Complete blood count + peripheral blood film

WBC/DC: 8,500/mm3 (4,600-11,000) N: 69%, L: 24% Hb: 8.5 g/dL (11-18) Hct: 29% (35-45) MCV: 104 fL (75-95) MCH: 39 pg (27-33) RDW: 19% (11.6-14.6) Plt: 227,000/mm3 (150,000-450,000) The blood film shows mild anisocytosis and macrocytosis, hypogranular and hypolobulated neutrophils, and 2% blast cells. No Auer rods are present in the blasts.

Reticulocyte count

Reticulocyte count: 1.4% (0.5-1.5)

Bone marrow aspiration and biopsy

The marrow is hypercellular, with dysplastic features involving erythroid and myeloid lineages. No Auer rods are present in the blasts. There are ~8% CD34+ cells. Ring sideroblasts are absent. Cytogenetic studies reveal the 11q deletion.

Serum erythropoietin

Serum erythropoietin: 280 mU/mL (4-19.5)


Step 3: Select appropriate management

Recombinant erythropoietin
Lenalidomide
Chronic red blood cell transfusions
Chemotherapy


Score: ★★☆