Contributed by Wen-Wei Chung, M.D., Ph.D., A. Julio Martinez, M.D.,
Susanne M. Gollin, Ph.D., Sofia Shekhter-Levin, M.D., Ph.D., Lydia Contis, M.D.
Published on line in August 1999
This 67 year-old male had a 3-year history of rheumatoid arthritis initially treated with gold and later with methotrexate. He presented with intermittent fever associated with chills, shortness of breath, difficulty in speech, back pain, lower extremity cramping and weakness. He had lost 15 pounds in a few months prior to admission. EKG showed atrial fibrillation, and the chest X-ray revealed pulmonary edema. Multiple computerized tomography (CT) scanning studies of chest, abdomen, and pelvis revealed moderate infiltrates in the upper segment of lower lobe of the right lung, and a single gallstone. Otherwise, CT scan studies were negative. No lymphadenopathy was identified. CT scan of the head revealed ventricular dilatation with central atrophy consistent with the patient's stated age of 67. No hemorrhage or mass lesion was identified. There was structural obscuring in the left basal ganglia suggesting early infarction. Peripheral blood findings were as follows (Table 1):
|Complete Blood Count||Patient Value||Normal Range (Male / Female)|
|White Blood Cells||7.1 x 109/L||4.5 - 11.0|
|Red Blood Cells||3.66 x 1012/L||4.35 - 5.87 / 3.79 - 5.23|
|Hemoglobin||9.9 g/dl||13.3 - 17.7 / 11.7 - 15.7|
|Hematocrit||29.7 %||40.0 - 52.0 / 35.0 - 47.0|
|Mean Corpuscular Volume||81.1 fl||80.5 - 99.7 / 80.0 - 100.0|
|Mean Corpuscular Hemoglobin||27 fl||28.2 - 32.2|
|Mean Corpuscular Hemoglobin Concentration||33.2 gm/dl||31.7 - 35.7|
|Red cell Distribution Width||19.1 %||11.5 - 15.0|
|Platelet count||259 x109/L||150-450|
The intermittent fever persisted; multiple blood and sputum cultures were negative. He received multiple antibiotics empirically. Polymerase chain reaction-based tests on cerebrospinal fluid for herpes simplex virus and cytomegalovirus DNA were negative. The erythrocyte sedimentation rate was markedly increased to 101 mm/h. Rheumatoid factor was elevated to 187 U/ml and antinuclear antibody titer was 1:80. He also had mildly elevated serum liver enzymes and progressively increased blood urea nitrogen and creatinine. He developed deep vein thrombosis in spite of Coumadin and heparin treatment. He thus received intravenous filter placement.
Two cytologic examinations of cerebrospinal fluid were negative for the presence of malignant cells. He also underwent biopsies of bone marrow, sural nerve, and skeletal muscle. A peripheral blood smear was submitted to pathology for evaluation of atypical cells (Figs 1, 2).