There was scanty amount of pericardial effusion without definite evidence of constriction. Open in a separate window Fig. to each other by virtue of their development from common progenitors in the B lymphocyte lineage. The clinical manifestations of MG relate to the expansion of the neoplastic cells, to the secretion of cell products, and to some extent to the host’s response to the tumor (1, 2). There have been numerous reports of multiple myeloma and related disorders with systemic amyloidosis associated with heart involvement (3-7). However, cardiac dysfunction associated with MG has scarcely been reported. We report a case of cardiac dysfunction associated with MG in which the diagnosis was made by echocardiography and biopsy. CASE REPORT A 59-yr-old man visited outpatient clinic for evaluation of progressive dyspnea on exertion and chest discomfort for 4 months. He had no remarkable medical and family history. On the physical examination, his height was 170 cm, weight 77 kg, the blood pressure 104/66 mmHg, the heart rate 76/min, the respiratory rate 20/min, and the body temperature 36. He had clear mentality, chronic ill-looking appearance and clear sclerae. Jugular venous distension was noted, but no cervical and axial lymphadenopathy was observed. The heart sounds were regular without murmur, but presystolic gallop (S4) was checked. The breath sounds were clear without rales or wheezing. Abdominal examination revealed normal bowel sounds without any palpable mass. There was no definite pitting edema. Otherwise the physical findings were unremarkable. The blood cell counts were as follows: the hemoglobin 13.0 gm/dL, the white blood cell count 6,700/L, and the platelet count 198,000/L. According to the serological biochemical assay, fasting blood sugar was 107 mg/dL, Na+ 142 mEq/L, K+ 4.0 mEq/L, Ca2+ 9.6 mg/dL, P- 3.7 mg/dL, blood urea nitrogen 12 mg/dL, creatinine 0.9 mg/dL, total protein 7.0 g/dL, albumin 4.3 g/dL, aspartate transaminase 20 IU/L, alanine transaminase 22 IU/L, alkaline phosphatase 188 IU/L, lactate dehydrogenase 285 IU/L, and beta-2 microglobulin 1.7 mg/L (normal; 0.8-2.2). Urinalysis showed trace amount of proteinuria, and the urine Bence-Jones protein was suspected. Serum protein electrophoresis study was normal. On the immunochemistry findings, IgG was 1,052 mg/dL (normal; 700-1,600), IgA 105 mg/dL (normal; 70-400), IgM 49 mg/dL (normal; 40-230), and IgE was elevated as 385 IU/mL (normal; 10-180). In serum SRPKIN-1 and urine immunoelectrophoresis study, lambda type MG was shown (Fig. 1). Open in a separate window Fig. 1 In serum (left) and urine (right) immunoelectrophoresis study, lambda type monoclonal gammopathy was shown. The chest radiography film showed a slightly enlarged cardiac silhouette and there was no pulmonary congestion. There was no bony lesion on plain skull radiography. Electrocardiography (ECG) showed regular sinus rhythm, intermittent atrial premature beats, poor R wave progression, and Q wave in precordial leads (Fig. 2). Open in a separate window Fig. 2 Electrocardiogram demonstrated regular sinus rhythm with premature atrial TRADD beat, left axis deviation, and poor R progression on precordial leads. The echocardiography revealed dilated both atria, concentric left ventricular (LV) hypertrophy (end-diastolic interventricular septum thickness=13 mm and end-diastolic posterior wall thickness=13 mm, respectively). Both LV dimension and LV ejection fraction were within normal limits. On the Doppler study, ratio of early mitral inflow (E) to late filling velocity (A) (E/A) was 3.0, deceleration time was 135 msec. Tissue Doppler showed 4.03 cm/sec of early diastolic mitral annular velocity (E’) with E/E’ ratio of 21.6, suggesting SRPKIN-1 restrictive physiology of diastolic dysfunction with elevated LV filling pressure (Fig. 3). There was scanty amount of pericardial effusion without definite evidence of constriction. Open in a separate window Fig. 3 Echocardiography demonstrated increased thickness of the left ventricle (LV) wall and both atrial enlargement and normal systolic function with normal wall motion. Mitral inflow and mitral anular Doppler tissue velocities showed grade 3 diastolic dysfunction and high E/E’, suggesting SRPKIN-1 markedly elevated LV filling pressure. Bone marrow (BM) aspirate smears showed normocellular marrow particle, and estimated M:E ratio was 1.71:1. The granulocyte series were normal in number and showed good maturation sequences. Plasma cells were increased and counted up to 6.8% of absolute neutrophil count compatible with monoclonal gammopathy of undetermined significance (MGUS) (Fig. 4). There were no amorphous eosinophilic amyloid material in both H&E and Congo-red staining for ruling out SRPKIN-1 amyloidosis for endomyocardial (Fig. 5A), BM, and enteral biopsies. However, immunostaining for lambda light chain in myocardium showed positive result (Fig. 5B). Open in a separate window Fig. 4 In bone marrow biopsy, plasma cells were increased and counted up.