A 44-year-old man presented to our emergency department with a fever, and feeling of dyspnea that had persisted for one month. He was a chronic alcoholic, and diagnosed with megaloblastic anemia 5 years earlier, but he never visited a hospital since then. He was acutely ill, with blood pressure of 110/66 mm Hg, pulse rate of 126 beats per minute, body temperature of 38℃, respiratory rate of 24 breaths per minute, and oxygen saturation of 94% at room air. Crackles were present in both lungs, and pitting edema was observed in both legs. Chest radiography and chest computed tomography (CT) showed bilateral multiple wedge shaped consolidations in a subpleural area, with a small amount of pleural effusion (
Figure 1).
Laboratory tests revealed a white cell count of 7,600/µL (neutrophil, 74%; eosinophil, 0.7%), hemoglobin concentration of 5.6 g/dL (reticulocyte, 1.2%; mean corpuscular volume, 114 µm
2), platelet count of 182,000/µL, erythrocyte sedimentation rate of 46 mm/hr, and C-reactive protein level of 13.6 g/dL. Analysis of the patient's arterial blood gases indicated a PaO
2 of 58 mm Hg, PaCO
2 of 33 mm Hg, HCO
3 of 31 mm Hg, and SaO
2 of 94%. Results of liver and renal function tests, except aspartate aminotransferase (91 U/L), alanine aminotransferase (64 U/L), and bilirubin (0.6 mg/dL) were within normal range. Owing to the possibility of community-acquired pneumonia, a course of empirical antibiotics was initiated, and the patient received a transfusion of packed red blood cells to relieve symptomatic anemia. However, cultures for common bacteria, acid-fast bacilli, and fungi were all negative; a simple chest radiography became worsened; fever up to 40℃ persisted despite antibiotic therapy; and he developed multiple painful, erythematous, palpable rashes on both lower legs (
Figure 2). We performed a skin biopsy to differentiate such as a drug rash or a transfusion reaction, but microscopic examination revealed the presence of neutrophilic infiltration in perivascular and interstitial area, which was compatible with cutaneous leukocytoclastic vasculitis (
Figure 3). Percutaneous needle biopsy was followed to rule out organizing pneumonia or consolidative lung cancer including lymphoma. However, the results of the needle biopsy indicated necrotizing vasculitis with perivascular infiltration of neutrophils and lymphocytes, granuloma formation, and intraluminal fibrosis, which were compatible with a diagnosis of leukocytoclastic vasculitis (
Figure 4). Serologic tests were all negative for venereal disease, hepatitis B surface antigen, hepatitis B and C, human immunodeficiency virus antibodies, anti-nuclear antibody (ANA), anti-neutrophil cytoplasmic antibody (ANCA), rheumatoid factor (RF), and cryoglobulin. There was no specific finding in abdominal CT scan. Since anemia persisted even after transfusion, we performed bone marrow aspiration with biopsy to identify the cause of unexplained macrocytic anemia. Evaluation of the bone marrow revealed hypercellularity (80%) that was abnormal for the his age, blast count of 2.6%, decreased erythropoiesis, and dysmegakaryopoiesis with karyotype of 46 XY,+1,der(1;7)(q10;q10). Finally, he was diagnosed as MDS, especially type of refractory cytopenia with multilineage dysplasia.
Treatment of intravenous methylprednisolone (1 mg/kg) was started for immunologic manifestations, and fever subsided immediately. After 3 days, the patient's skin and lung lesions began to improve (
Figure 5A), and his anemia improved. Since the identified karyotype was associated with poor prognostic outcome which has potential of progress to acute leukemia, we recommended him to undergo allohematopoietic stem cell transplantation (HSCT) as treatment for MDS. He refused our suggestion; however, and insisted on being discharged from our hospital, so we prescribed him an oral corticosteroid for 1 month, serially tapering the dosage every week.
After 1 year, he returned to our emergency department complaining of left flank pain. CT scans of the patient's abdomen revealed splenomegaly, and leukemic transformation was suspected on peripheral blood smear. His chest radiograph, however, showed improvement of pulmonary vasculitis compared to 1 year before, despite no further therapeutic intervention (
Figure 5B). We then transferred him to another facility to undergo bone marrow transplantation.