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Hyperviscosity Syndrome Complicating Rheumatoid Arthritis: Report of Two Additional Cases and Review of the Literature

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Hyperviscosity Syndrome Complicating Rheumatoid Arthritis: Report of Two Additional Cases and Review of the Literature Henry Ford Hospital Medical Journal Volume 30 Number 4 Article 3 12-1982 Hyperviscosity Syndrome Complicating Rheumatoid Arthritis: Report of Two Additional Cases and Review of the Literature Michael R. Lovy Mark C. Kranc Gilbert B. Bluhm Jeanne M. Riddle Paul D. Stein Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Lovy, Michael R.; Kranc, Mark C.; Bluhm, Gilbert B.; Riddle, Jeanne M.; and Stein, Paul D. (1982) "Hyperviscosity Syndrome Complicating Rheumatoid Arthritis: Report of Two Additional Cases and Review of the Literature," Henry Ford Hospital Medical Journal : Vol. 30 : No. 4 , 189-198. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol30/iss4/3 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Henry Ford Hosp Med J Vol 30, No 4,1982 Clinical Reports Hyperviscosity Syndrome Complicating Rheumatoid Arthritis: Report of Two Additional Cases and Review of the Literature Michael R. Lovy, MD,* Mark C. Krane, MD,** Gilbert B. Bluhm, MD, *** Jeanne M. Riddle, PhD,*** and Paul D. Stein, MD**** A hyperviscosity syndrome developed in two patients ity caused by the presence ofthe high molecular weight with rheumatoid arthritis. Analytic ultracentrifugation complexes. These abnormalities, as well as the clinical of the sera from one patient demonstrated 225 com­ bleeding, whole blood, and plasma viscosity, became plexes. Intermediate, 22S, and 375 complexes were normal after treatment. In vivo leukocytic function was found in the second case. Platelet aggregometry and studied with the Rebuck skin window technique, and electron microscopy surveys of the platelet reactivity in phagosomal inclusions were observed in both the poly­ one patient demonstrated inhibition of platelet reactiv­ morphonuclear neutrophils and macrophages. Hyperviscosity syndrome is characterized by abnor­ microscopic studies of the platelet reactivity suggested malities affecting multiple organ systems. Retinal hem­ that intermediate and high molecular weight proteins orrhages, congestive heart failure, neurologic and hemo­ were capable of altering platelet morphology and inter­ static abnormalities, and symptoms of anorexia, fatigue, fering with in vitro platelet aggregation. and malaise are its manifestations. Although hyper­ gammaglobulinemia and elevated serum viscosity fre­ Case Reports quently occur in patients with rheumatoid arthritis (1), they rarely reach levels necessary to produce hypervis­ Case 1 cosity syndrome. A 43-year-old black woman was admitted to Henry Ford Hospi­ tal in April 1969 because of anorexia, weight loss, fatigue, and Among the reported cases of hyperviscosity syndrome frequent nosebleeds. She had been well until 1966, when she complicating rheumatoid arthritis (2-8), the specific developed rheumatoid arthritis. She had numerous extra­ immunoglobulin abnormality causing the increased vis­ articular manifestations of rheumatoid arthritis, including sub­ cosity has varied. These abnormalities include polymers cutaneous nodules, leg ulcers, a rheumatoid pleural effusion, of IgG, which showed rheumatoid factor activity and and Sjogren's syndrome. cryoglobulin properties (2), IgG rheumatoid factor cir­ On examination, there were decreased breath sounds at the culating as a polyclonal 13S component (4), intermediate base of the right lung, but no signs of active synovitis. Labora­ complexes formed by self-association of IgG rheuma­ tory results included the following: hemoglobin 11.3 gm%, toid factor molecules (6), and interaction of IgM rheu­ white blood cell count 4,000/mm3, with 44% polymorphonu­ matoid factor with intermediate complexes (3,7). clear cells, 3% eosinophils, 1% basophils, 15% lymphocytes. We have observed two additional patients with a hyper­ viscosity syndrome complicating rheumatoid arthritis. Submitted for publication: March 1,1982 Accepled for publication: May 13,1982 By the use of analytic ultracentrifugation, we were able •Preseni Address: 1901 Cedar, Suite 201, Tacoma, Washington 98405 to demonstrate 22S complexes in the sera of one patient ••2570 Haymaker Rd, Monroeville, Pennsylvania 15146 and high molecular weight complexes in the other. •••Department of Internal Medicine, Rheumatology Division, Henry Ford Hospilal These complexes were responsible for the hyperviscos­ ••••Department of Internal Medicine, Division of Cardiovascular Medicine, ity. The bleeding diathesis was studied in each patient. I n Henry Ford Hospilal Address reprints to Dr. Bluhm, Henry Ford Hospilal,2799W Grand Blvd, Detroit, the second patient, platelet aggregation and electron Ml 48202 Lovy, Krane, Bluhm, Riddle, and Stein 32% atypical lymphocytes, and 5% monocytes. The erythro­ cytes showed marked rouleaux formation and numerous SKIN ULCER HYPERVISCOSrTY SJOGRENS SYNDROME PERICARDIAL FRICTION RUB target cells. The platelet count was 442,000/mm^. The relative POLYARTHRmS PLEURAL CHF serum viscosity was 6.0 (normal 1.2-2) (9). Other laboratory •ik_-l I—1EFFUSION parameters included: erythrocyte sedimentation rate (ESR) Imm/hr (Wintrobe method), lupus erythematosus (L.E.) cell TOTAL preparation positive, strong homogeneous antinuclear factor, PROTEIN sodium 131 mEq/1, chloride 103 mEq/1, and C02 combining Cg/100 ml) power 23 mEq/1, total protein 8.8 g/dl, gamma globulin 5.0 g/dl, IgG 4,640 mg/dl, IgM 2,347 mg/dl, and IgA 280 mg/dl. GAMMA Examination of the bone marrow aspiration showed lympho­ GLOBULIN ' cytosis, plasmacytosis, and slight eosinophilia. The following Cg/100 ml) ' SERUM VISCOSITY tests were either negative or normal: rheumatoid factor CYCLOPHOS-'" PHAMIDE ^" agglutination titer (10), urinalysis, metastatic survey, 24-hour (mg/day) w urine for protein and immunoglobulin electrophoresis, serum PREDNISONE ^• (mg/day) cryoglobulins, sickle cell preparation, VDRL, total hemolytic NOV APBIL \ M*y I complement, acute and convalescent viral antibody titers for polio types 13, Echo 6, 9, and Coxsackie A-9, Bl, 85, alkaline Fig.1 phosphatase, SCOT, and BSP retention. A survey of the plasma Clinical and laboratory course of Case 1. DRAT = differential rheuma­ coagulation system showed that the Lee-White and plasma toid agglutination titer. Normal range for relative serum viscosity is clotting time, fibrin stabilization factor, tests for circulating 1.2-2. anticoagulants, and factor IX assay were normal. Before the patient received any therapy, coagulation studies showed exertion, orthopnea, weakness, malaise, bleeding from his abnormal results, with a bleeding time of eight minutes, acti­ gums of two months' duration, and a weight lossof 15 pounds. vated partial thromboplastin time (APTT) of 98 seconds (con­ In the month before he was admitted, he had noted increased trol: 64 seconds), poor clot retraction, and a two-stage PIT morning stiffness and pain with swelling in his joints. assay for factor VIII of 74%. He was in no acute distress, afebrile, his blood pressure was During her hospitalization, she developed bleeding of the 140/90 mm Hg, and he had a regular pulse of 100/min. His gums, and cyclophosphamide (150 mg/day) was given orally. cheeks were erythematous. The retinal veins were distended, One week later, when she developed a pericardial friction rub and there were scattered hemorrhages and exudates. He had and congestive heart failure, therapy with prednisone (60 only limited movement of his jaw. He had bilateral contrac­ mg/day) was added to the treatment regimen. Within one tures with crepitation and synovial thickening of the elbows week she showed improvement of the congestive heart fail­ and wrists, ulnar deviation of the hands with symmetrical ure, her appetite returned, and bleeding ceased. Coagulation deformity of the metacarpal phalangeal and proximal inter­ studies were normal three weeks after the cyclosphosphamide phalangeal joints, and a moderate effusion in the right knee. medication had been initiated. No subcutaneous nodules were present. By June 1969, the patient was doing well on prednisone 10 The hemoglobin was 9.5 gm%, the white blood cell count mg/day and cyclosphosphamide 50 mg every other day. The 5,800/mm^ with a normal differential count except for an relative serum viscosity (11-13) was 1.75, total protein 6.8 occasional atypical lymphocyte and mononuclear cells resem­ gm/dl, gamma globulin 1.6 g/dl, IgC 1,244 mg/dl, IgM 384 bling Mott cells (Fig. 2A). The platelet count was201,000/mm3. mg/dl, IgA 153 mg/dl (14), and rheumatoid factor titer 1:320 The erythrocytes showed moderate rouleaux formation. Rheu­ (15). Subsequent doses of medications and corresponding matoid factor titer was 1:20,480 (15). Laboratory results are laboratory results were correlated (Fig. 1). summarized in Tables I and 11. Serum protein electrophoresis Rheumatoid erosions ofthe interphalangeal joints, as demon­ demonstrated a gamma globulin peak of 5.9 g/dl with re­ strated radiographically, and rheumatoid factor activity in the stricted mobility. A 24-hour urine collection contained a trace synovial fluid were subsequently shown. Over the following amount of free kappa chains, but no Bence-Jones protein. eight years, she had infrequent flares of arthritis. Treatment Bone marrow aspiration showed an atypical lymphocytosis and with indomethacin, aspirin,
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