Case 2
A 63-year-old gentleman, known to have hypertension was referred to hematology clinic in November 2018, after he was detected to have high hemoglobin (20.3 mg/dl, normal < 16.5 mg/dl) with high hematocrit (62.6%, normal 35% to 45%), erythrocytes (7.2 x106/µL, normal 3.8 to 4.8), leukocytes (14.3 x103/µL, normal 4 to 10) and thrombocytes (674 x103/µL, normal 150 to 400). A blood smear showed erythrocytosis with normal indices with a packed smear appearance, neutrophilic leukocytosis, and marked thrombocytosis. Ultrasound of the abdomen showed that the liver measured 14.4 cm, and the spleen measured 15.4 cm (Fig.2.). He was positive for JAK2V617F mutation and was diagnosed with PV. Treatment was initiated with hydroxyurea 1 gram daily. Six months later, he was found to have pancytopenia during his routine follow-up. He had a severely reduced hemoglobin of 2.9 mg/ dl, a white cell count of 2.3 x103/µL, and a platelet count of 69 x103/µL. He also complained of diplopia when looking towards the left for a week. Peripheral smear showed no blasts, and repeated ultrasound showed a decrease in the size of the spleen. Hydroxyurea was stopped under the impression of drug-induced bone marrow suppression; supportive transfusions were given, and blood counts were monitored. The neurological evaluation showed that he had diplopia that becomes more pronounced on the left lateral gaze. There was a bilateral restriction of adduction and vertical movements of eyes. Pupils were equal and responsive to light. There was no facial asymmetry, and the gag reflex was preserved. Left-sided ptosis was noted, which worsened with repeated movements of the eyelid. He developed hypophonia after counting out loud. The patient’s son added that he has been having a deconjugate gaze and generalized weakness towards the end of the day for the last two years, but these symptoms were ignored. There was a high clinical suspicion of MG, and he was started on pyridostigmine 60 mg daily, observing for clinical response. There was an improvement in left eye ptosis over the next three days, and he was maintained on the same dose of pyridostigmine. AchR antibody was positive, but there was no thymoma on computed tomography (CT) of the thorax. Hydroxyurea was stopped, and he was maintained on close follow-ups to monitor blood counts. Therapeutic venesection was done as and when needed. He was asymptomatic from the MG point of view and had normal blood counts during his latest clinic visit in June 2020.