A 33-year-old-female presented with progressive exertional dyspnea and bilateral lower extremity edema for 1 week associated with orthopnea, palpitation & fatigue. She also had 3 kilograms of weight loss in 2 months with normal appetite. She was anxious, looked very pale and in mild respiratory distress. Pulse was 113 /min regular, temperature 37.9 celsius, blood pressure 100/60 mmHg, respiratory rate 26/minute and oxygen saturation was 98 % on 2 liter/min of oxygen. She was jaundiced with pale face & palmar creases. Her palms were sweaty with warm extremities and bilateral pitting lower limb edema and elevated jugular venous pulse. There was bilateral diffusely enlarged thyroid with bilateral chemosis, lid retraction & exophthalmos (proptosis). Cardiopulmonary exam showed S3 gallop & bilateral basal fine inspiratory crackles with normal intensity of breath sounds.
Complete blood count revealed pancytopenia with hemoglobin 5.4 g/dl, MCV 104 fl, WBC 2.7 (absolute neutrophil count of 1.3) and platelets 78,000/mm3. Peripheral smear showed polychromasia with severe spherocytosis and hemolysis work up was positive including very low haptoglobin and strongly positive direct anti-globulin test (IgG). TSH was < 0.01 mIU/l (Normal: 0.45-4.5 mIU/l), Free T4 51 pmol/l (Normal: 9-20 pmol/l) and Free T3 40 pmol/l (Normal: 3.4-6 pmol/l). Thyroid Stimulating Immunoglobulins (TSI) was positive. Chest x-ray showed pulmonary edema. Echo was normal with ejection fraction 65%. CT chest, abdomen and pelvis was unremarkable. Bone marrow biopsy was hypercellular without any abnormal cells.
She was treated with diuresis, blood transfusion, propranolol and methimazole. Her blood counts recovered in 2 weeks after starting anti-thyroid medication (methimazole) without requiring steroids. Based on these findings it was concluded that acute high output heart failure was precipitated by Graves’ disease and autoimmune hemolytic anemia. Moreover, autoimmune pancytopenia was most likely associated with Graves’ disease.
Discussion:
Graves’ disease is the most common cause of hyperthyroidism and it is associated with various autoimmune diseases. However, it is rarely reported to be associated with autoimmune pancytopenia. Acute high output heart failure as first clinical manifestation in setting of Graves’ disease and autoimmune hemolytic anemia is also uncommon. In this case, improvement in pancytopenia with use of antithyroid medications without use of steroids points towards autoimmune etiology.
Conclusions:
Patients with Graves’ disease may present with uncommon clinical manifestations and must be carefully and timely evaluated for other autoimmune associations including autoimmune pancytopenia. Antithyroid medications alone can be effective in treatment of autoimmune pancytopenia in setting of Graves’ disease.