Hemarthrosis as the presenting manifestation of true myeloma joint disease

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148 CASE REPORT HEMARTHROSIS AS THE PRESENTING MANIFESTATION OF TRUE MYELOMA JOINT DISEASE GREGORIO MINTZ, ELSA J. ROBLES-SAAVEDRA, RUBEN D. ENRfQUEZ, F. JAVIER JIMENEZ, and MA. LOURDES JUAN This is the first report of a patient with hem- arthrosis due to invasion of the synovial membrane by myeloma cells. With angiographic studies of the affected joint it was apparent that the tumor tissue extended from the destructive bone lesion of the femoral condyle into the synovial membrane. Intraarticular spontaneous bleeding was the first manifestation of a monoclonal IgG multiple myeloma; the hemarthrosis recurred after drainage but was controlled with local roentgen therapy. Multiple myeloma is a malignant disease of plasma cells; even though the skeletal system is the seat of early and common clinical manifestations, the joints do not usually become involved with this pathologic process. Amyloid arthritis is the recognized type of joint disease in these patients (1-2). From the Department of Rheumatology. Hospital General del Centro Medico Nacional. lnstituto Mexican0 del Seguro Social, Mexico City, Mexico. Gregorio Mintz, M.D.: Professor and Chairman, Depart- ment of Rheumatology: Elsa J. Robles-Saavedra, M.D.: Associate, Department of Rheumatology; Rubtn D. Enriquez, M.D.: Associate, Department of Rheumatology; F. Javier Jimtnez, M.D.: Associate, Department of Rheumatology; Ma. Lourdes Ju in , M.D.: Resident, Department of Rheumatology (present address: Hospital Colonia, Mexico City). Address reprint requests to Gregorio Mintz. M. D., Hospital General del Centro Mtdico Nacional. IMSS. Ave. Cuauhttmoc 330, Mexico 7. D. F. Submitted for publication June 2, 1977: accepted July 20, 1977. This report describes a patient with myeloma cell infiltrate of the synovium and true myeloma arthritis that made its clinical debut as a hemarthrosis, condi- tions not previously recognized in multiple myeloma. CASE REPORTS A 69-year-old Mexican male was admitted with a 6- month history of knee pain, swelling, low back pain, and weight loss. Physical examination revealed a chronically ill, pale patient with marked swelling of the left knee and quad- riceps atrophy, and limitation of back motion with percussion tenderness in the low back. Table 1. Laboratory Findings Hemoglobulin 8.7 grams/dl Hematocrit 26% White blood cells 6900/mm3 Nonprotein nitrogen 109 mg/dl Creatinine 3.6 mg/dl Calcium 12.7 mg/dl Uric acid 18 mg/dl Total proteins 9.4 grams/dl Albumin 3.02 grams/dl Alpha-I-globulin 0.37 gram/dl Alpha-2-globulin 0.67 gram/dl Beta-globulin I .32 grams/dl Gamma-globulin (monoclonal pattern) 4.02 grams/dl IgG 3580 mg/dl Proteinuria 8.6 grams/liter Bence Jones test Strongly positive Arthritis and Rheumatism. Vol. 21, No. I (January-February 1978) HEMARTHROSIS IN MYELOMA JOINT DISEASE 149 Pertinent laboratory findings are summarized in Table I . Radiograph studies revealed multiple lytic lesions of the skull pelvis and left lateral femoral condyle (Figure I ) . A sternal bone marrow aspirate showed 55% abnormal plasma cells and plasmoblasts diagnostic of multiple myeloma. An arthrocentesis was performed with a 19-gauge needle and 250 ml of grossly bloody fluid were removed. This fluid had a hematocrit of 20% with a monoclonal protein electrophoretic pattern identical to the one obtained in the serum. Parker-Pearson needle biopsies were obtained from both knees; the right knee synovial membrane had a normal histology and the left side showed a dense infiltrate of the synovial tissues with myeloma cells (Figure 2) and increased vascularization. No amyloid was found with the special stain- ing techniques. A left femoral arteriograph (Figure 3) showed the lateral superior geniculate artery with abnormally increased circulation entering the area of bone destruction and produc- ing a typical tumor blush. The patient received multiple chemotherapy with pred- nisone 80 mg/day, melphalan I mg/kg divided in 4 days, cyclophosphamide 2 mg/kg/day, and Bcnu 150 mg i.v., plus roentgen therapy to the knee (total dose: 4,000 roentgens). The synovial effusion subsided slowly during 5 weeks; after 1 year there was new bone formation that filled again the destructive condyle lesion. Two years later the patient died in renal failure. DISCUSSION There are several rheumatic manifestations o f multiple myeloma. The most frequently encountered (2) is bone pain usually localized at the site of the osteolytic lesions. Because the vertebral column is a common lo- calization o f multiple myeloma, it is always considered in the differential diagnosis of low back pain in the elderly. Bone tumors and pathologic fractures also oc- cur as presenting symptoms o f multiple myeloma. Figure I A. Anreroposterior radiograph of the left knee showing a large desrrucrioe bone lesion in rhe lateral femoral condyle. Figure I B. The patella is outlined with a dotted line and the bone lesion is depicrrd behind it. 150 MINTZ ET AL Amyloid arthritis is the commonly recognized joint disease in multiple myeloma (1) . Deposit of amy- loid material can be found in the synovium, producing enlarged and painful joints, and also in the paraarticular tissues, giving rise to a carpal tunnel syndrome (3). A polyarticular form has also been described which clini- cally resembles rheumatoid arthritis (3-4), including swelling and involvement of the small joints of the hands. This polyarthritis frequently precedes any overt manifestation of myeloma, but it may develop later in the course of the disease. Gout (2 ,5 ) has not been commonly described in multiple myeloma. Because of the frequent hyper- uricemia in these patients, one might suspect a higher incidence than is recognized at present. Septic arthritis due to the abnormal humoral im- Figure 3A. Femoral arteriography. The femoral and popliteal ar- teries and their colaterals. including the capsular artery of the knee, are all normal. munity that accompanies the production of abnormal serum proteins in multiple myeloma (6) has been re- ported, and necrotizing arteritis has been recognized in one instance (7). Myelomatous infiltration of the synovium has not been previously described. It was an early manifesta- tion of disease in our patient, and we believe that it was a direct invasion to the synovial membrane from the de- structive lesion of the femoral condyle. Hemarthrosis is a recognized manifestation of the hereditary hemorrhagic disorders, classic hemo- philia and deficiency of other clotting factors (1); trauma (8); and tumors or tumor-like conditions includ- ing pigmented villonodular synovitis (9) and synovial hemangiomas (9,lO). It has been described with less frequency with metastatic tumors, metallic arthroplasty, Figure 2. Microscopic appearance of the synovial membrane of the IeJl knee. There is a dense infiltrate of a synovial villi with large ab- normal plasma cells. (Hematoxylin and eosin stain. original mag- nifcation X 4 0 0 ) . HEMARTHROSIS IN MYELOMA JOINT DISEASE 151 Figure 38. The lateral superior geniculare artery (arrow) is increased in size and runs toward the destructive condyle. The artery gives origin to abnormal colateral neo$ormed circulation (arrowheads) with tortuous arreries indicating excessive flow in an area of oval-shaped tumor tissue. osteoarthritis, chondrocalcinosis, amyloidosis, sickle cell disease, rheumatoid arthritis, infection, and neu- ropathic joint disease, and as a result of anticoagulant therapy ( 1 1). An additional report of hemarthrosis in myeloproliferative disease associates it with thrombocy- tosis and malfunction of platelets (12). This is the first report of true myeloma joint disease, not amyloid arthritis, in a patient with mono- clonal IgG multiple myeloma. Although infrequent, multiple myeloma should be added to the list of condi- tions capable of producing spontaneous intraarticular bleeding. REFERENCES 1. Rodnan GP: Arthritis associated with hematologic dis- orders, storage diseases and dysproteinemias, Arthritis and Allied Conditions, Eighth edition. Edited by J L Hol- lander and DJ McCarty, Jr. Philadelphia, Lea & Febiger, 2. Hamilton EBD, Bywaters EGL: Joint symptoms in myelo- matosis and similar conditions. Ann Rheum Dis 3. Tarr L, Ferris HW: Multiple myeloma. Arch Intern Med 4. Davis JS, Weber FC, Bartfeld H: Conditions involving the hemopoietic system resulting in a pseudorheumatoid ar- thritis: similarity of multiple myeloma and rheumatoid arthritis. Ann Intern Med 47:lO-17, 1957 5 . Bronsky D, Bernstein A: Acute gout secondary to multiple myeloma: a case report. Ann Intern Med 41:820-823, 1954 6. Fahey JL, Scoogins R, Utz JP, Szwed CF: Infection, anti- body response and gammaglobulin components in mul- tiple myeloma and macroglobulinemia. Am J Med 7. Skoog WA, Adams WS: Metabolic balance study of a patient with multiple myeloma treated with dexamata- sone. Am J Med 34:417-425, 1963 8. Jessar RA: The study of synovial fluid, Arthritis and Al- lied Conditions. Eighth edition. Edited by J L Hollander, DJ McCarty, Jr. Philadelphia, Lea & Febiger 1972, pp 9. Cohen AS: Tumors of synovial joints, bursae and tendon sheaths, Arthritis and Allied Conditions. Eighth edition. Edited by J L Hollander, DJ McCarty Jr. Philadelphia, Lea & Febiger 1972, pp 1374-1387 10. Stevens J , Katz PL, Archer FL, McCarty DJ: Synovial hemangioma of the knee. Arthritis Rheum 12:647-649, 1969 I I . Wild JH, Zvaifler NJ: Hemarthrosis associated with so- dium warfarin therapy, Arthritis Rheum 19:98-102, 1976 12. Harris BK, Ross HA: Hemarthrosis as the presenting manifestations of myeloproliferative disease. Arthritis Rheum 17:969-970, 1974 1972, pp 1303-1328 201353-362, 1961 641820-833, 1939 35:698-707, 1963 67-8 1


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