American Journal of Emergency Medicine (2005) 23, 811 - 812

Case Report

Emergency plasmapheresis for unstable angina in a patient with hyperviscosity syndrome

Shmouel Ovadia MD*, Lyudmila Lysyy MD, Sharon Floru MD

Department of Internal Medicine bC,Q E. Wolfson Medical Center, Halochamim 4 Holon, Israel

Received 5 March 2005; accepted 5 March 2005

Single case report

The subject is a 66-year-old man who underwent a left nephrectomy in 1976 because of Renal cell carcinoma. He is post-myocardial infarction, presently with stable ischemic heart disease, hypertension, and anemia from vitamin B₁₂ deficiency. Two weeks before admission, the subject reported weakness, lack of appetite, and weight loss. A few days later, swelling of the testicles with no fever appeared. On admission, physical examination revealed bilateral epididymal sensitivity. Electrocardiogram revealed normal sinus rhythm with first degree atrioventricular block. Examination by a urologist produced a diagnosis of acute bilateral infection of the epididymis.

In the course of hospitalization, he was treated with cefuroxime and ciprofloxacin, with gradual improvement in his condition. Notable in the course of his hospitalization were polyclonal hypergammaglobulinemia and a relative serum viscosity of more than 4.0 (normal, 1.4-1.8). On urine testing, all of the serum proteins and partial monoclonal bands were observed. Because of the results, the following were performed: bone x-rays, which were normal, and bone marrow biopsy, in which were found lymphoid infiltrates, mostly formed of small cells. On immunohistochemical coloring, a polyclonal increase of plasma cells was found, colored to both j and k and to different immunoglobulins. The cells were positively colored for CD20. On consultation

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with the hematologic unit, lymphoproliferative disease was ruled out.

A week after the bone marrow biopsy, the patient began to report swelling and pain in the area of the right parotid gland. On examination by an ear, nose, and throat physician and ultrasound of the affected gland, Acute infection of the right parotid gland was diagnosed. Ultrasound revealed a homogeneous structure of the gland, with a number of lymph nodes in and around the gland. At this point, with the possible diagnosis of Sjogren syndrome, Schirmer test was performed, which was found to be regular, with no dryness. Biopsy of the small salivary glands of the lower lip revealed periductal and perivesicular infiltration of lymphocytes, a histologic picture typical of Sjogren syndrome.

To support the diagnosis, additional antibody tests were performed: anti-Ro (SS-A) was 398.30 U/mL and anti-La (SS-B) was 238.2 U/mL, which is considered highly positive (see Fig. 1).

The diagnosis of Sjogren syndrome was therefore made on the basis of a clinical picture, which included hemolytic anemia (positive Coombs test), involvement of exocrine glands (parotid and epididymal), a positive biopsy with involvement of the salivary glands, dryness of the mouth (xerostomia), and a sensation of dryness of the eyes in spite a negative Schirmer test. Furthermore, antibody tests were positive and supported the diagnosis of Sjogren syndrome. During hospitalization, the patient experienced severe chest pain, with anterior ST-segment changes on electrocar- diogram, which did not respond to standard treatment. The decision was made to administer 3 sessions of plasmaphe- resis, which resulted in symptom-free angina. Although it is

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812 S. Ovadia et al.

Fig. 1 Immunoglobulin levels before and after treatment with plasmapheresis. Note the drop in immunoglobulins after plasmapheresis on January 27, 2003.

not standard protocol to treat patients with Sjogren syndrome with plasmapheresis, this was done because of the patient’s deterioration due to his unstable angina as a result of his hyperviscosity syndrome. At 2-year follow-up, after con- tinuing treatment with Imuran and cyclophosphamide, the patient is still free of any ischemic or anginal symptoms.

Discussion

Hyperviscosity is an impairment in blood flow due to increased blood viscosity that can derive from an increase in the plasma proteins and red blood cells. Hyperviscosity can appear in diseases such as Waldenstrom macroglobulinemia, multiple myeloma, and autoimmune diseases [1]. Hypervis- cosity can result in visual abnormalities, neurologic damage, coagulopathies, and thrombosis. Sjogren syndrome can be expressed as hypergammaglobulinemia. The irregular rheo- logic characteristics of the serum result from the molecular structure of the monoclonal paraproteins, which can accumulate because of the formation of polymers. In these models, IgG behaves as an antigen and antibody and can undergo polymerization to a dimer and more complex models and molecules. Generally, medium chains are the cause of formation of these complexes.

To date, few cases of rheumatologic diseases with hyperviscosity, and even fewer cases of Sjogren syndrome

with hyperviscosity, have been reported [2- 4]. In only 1 case could we find a report on treatment with plasmaphe- resis. The treatment of rheumatoid diseases with plasma- pheresis is generally very limited and Sjogren syndrome is not considered an indication for the use of this therapy. Our attempt at treatment of the patient under discussion was successful, with no complications. As far as we know, this is the first case of Sjogren syndrome with unstable angina that was treated successfully with plasmapheresis. Follow-up over the course of 2 years has not indicated a need to repeat the treatment. We therefore believe that the use of plasmapheresis should be considered in the treatment of Sjogren syndrome with hyperviscosity and ischemic heart disease.

References

1. Hadler NM, Don GK, Chung S, et al. Polyclonal hyperviscosity syndrome. Arthritis Rheum 1977;20(7):1388 - 94.
2. Simon AJ, Lazo-Langner A, Duarte-Rojo A, et al. Serum hypervis- cosity syndrome responding to therapeutic plasmapheresis in a patient with primary Sjogren’s syndrome. J Clin Apheresis 2002;17:44 - 6.
3. Blaylock WM, Waller M, Normansell DE. Sjogren’s syndrome: hyperviscosity and intermediate complexes. Ann Intern Med 1974; 80:27 - 34.
4. Avnstorp C, Nielsen H, Drachmann O, et al. Plasmapheresis in hyperviscosity syndrome. Acta Med Scand 1985;217:133 - 7.