The patient to be discussed today is an 8 1 โ2-year-old girl. She was referred to us in February 1994 when 4 years old because of recurrent severe headaches, paraesthesias of the extremities, erythromelalgia, abdominal pain, and a platelet count of between 1,300 and 1,500 ร 10 9 /L. Physical examination was noncontributory. The ultrasonographic evaluation of the abdomen showed a slightly enlarged spleen. Electroencephalogram and cranial magnetic resonance imaging were normal. Professor Zintl, can you reconstruct for us the diagnostic possibilities that were considered at that time?
Felix Zintl, MD (Pediatric Oncologist, Director) Chronic myeloproliferative diseases, albeit very rare in children, enter the differential diagnosis; so it is entirely appropriate to review this patient in our Tumor Board. The most important question, actually, was to distinguish between secondary or reactive thrombocytosis and essential thrombocythemia (ET). The several laboratory investigations that were undertaken will now be revieved.
Axel Sauerbrey, MD (Pediatric Oncologist)
Hemoglobin concentration was 9.5 mmol/L (15.2 gm/ L), and the leukocyte count 14.4 ร 10 9 /L with 57% neutrophils, 31% lymphocytes, 8% eosinophils, and 4% monocytes. Platelet count was 1800 ร 10 9 /L. In the serum, we found a pseudohyperkalemia of 5.8 mmol/L. The alkaline phosphatase in neutrophils was within normal range.