Clinical picture Grave’s disease and acquired hyposplenism

A 38-year-old Caucasian woman was referred to the hematology clinic for unexplained leukocytosis and thrombocytosis and ‘possible myeloproliferative disorder’. Her history included only Grave’s disease (GD) in remission for 2 years after methimazole treatment. A recent blood count revealed hemoglobin content to be 12.8 g/dl; white blood cells 20.7 10 m/l (neutrophils 9.5 10; lymphocytes 8.3 10; monocytes 2.3 10; and eosinophils 0.5 10); and platelets 580 10 m/l. The patient denied fever, night sweats, weight loss or pruritus and had no other symptoms. Examination showed a marked exophthalmos (Figure 1) with normal skin, heart rate (82 beats/min), blood pressure (130/ 75mmHg) and no tremor. The thyroid was not tender but mildly and diffusely enlarged. The heart, lungs, breast and abdominal examination was normal with no organ enlargement. Both lower limbs showed marked nonpitting edema and ‘peaux d’orange’ appearance of the skin (Figure 2). Peripheral blood smear (PBS) examination revealed numerous Howell–Jolly bodies (Figure 3). Anisocytosis and poikilocytosis as well as marked neutrophilia, and moderate lymphocytosis, monocytosis and thrombocytosis were also seen. Biochemistry tests were normal including acutephase reactants, serum proteins and urinalysis. Thyroid-stimulating hormone level was 0.03 MIU/ ml (normal 0.34–5 MIU/ml); free T4 was 13 pmol/l (normal 7–17 pmol/l); and total T3 1.74 nmol/l (normal 1.34–2.73 nmol/l). Autoantibody screening revealed positive antinuclear antibodies (ANA), while antibodies to dsDNA, Ro (SS-A), La (SS-B), endomysium, tissue transglutaminase, gliadin and rheumatoid factors were found negative. Bone marrow histology showed moderately hypercellular marrow with no abnormal cells and no fibrosis. Polymerase chain reaction analysis was negative for BCR-ABL rearrangement and for JAK2 mutation. A liver–spleen Technetium scan showed no uptake in the spleen (Figure 4). An abdominal computed tomography (CT) scan demonstrated a very small Figure 2. The patient’s bilateral edema of legs with elephantiasis and ‘peaux d’orange’ appearance of the edematous skin. Figure 1. The patient’s bilateral exophthalmos.