Scandinavian Journal of Rheumatology

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Polymyalgia Rheumatica and Chronic Lymphatic Leukaemia Johan von Knorring & Olof Selroos To cite this article: Johan von Knorring & Olof Selroos (1977) Polymyalgia Rheumatica and Chronic Lymphatic Leukaemia, Scandinavian Journal of Rheumatology, 6:3, 145-147, DOI: 10.3109/03009747709095438 To link to this article:

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Date: 25 March 2016, At: 19:02

Scand J Rheumatology 6: 145-147, 1977


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From the Fourth Department of Medicine, Helsinki University Central Hospital, Helsinki, Finland

ABSTRACT. A patient with polymyalgia rheumatica (PMR) is reported in whom, at the time of diagnosis, the number of lymphocytes in peripheral blood and bone marrow was normal. Lymphocytic infiltration typical of PMR was detected in renal and muscle biopsy specimens, and a liver fine-needle aspirate contained an abnormal lymphocytic infiltration, probably leukaemic. The number of lymphocytes gradually increased in bone marrow and peripheral blood until, almost 5 years after PMR had been diagnosed, a typical picture of chronic lymphatic leukaemia (CLL) was observable. The significance of the reported combination is discussed. The possibility is pointed out that a CLL in progress must be suspected when persistent lymphocytosis occurs in a patient with typical PMR, even if initially the response to treatment with corticosteroids suggests that PMR is the sole disorder.

Lymphoid malignancy has been reported in rheumatoid arthritis and in systemic lupus erythematosus ( 2 ) . and an association between malignant lymphomas and other reticuloses and various rheumatic diseases has been confirmed (12). Patients with malignant lymphoma or chronic lymphatic leukaemia (CLL) may have a variety of musculoskeletal symptoms but joint involvement is unusual (5). A previous report from our hospital described a patient with typical polymyalgia rheumatica (PMR), in whom a malignant lymphoma developed (8). Of our series of 51 patients with either PMR or temporal arteritis (TA), CLL developed in another patient who had classical PMR of long duration. His case history forms the basis of this report.

CASE REPORT The patient, a 76-year-old man, was admitted to another hospital in August 1970 because of muscle pains and morning stiffness in his shoulders. The clinical course is graphically presented in Fig. I . His erythrocyte sedimentation 10- 77 1863

rate (ESR) was 90-100 mm/h, Hb 10.5 gll00 ml, and the leukocyte count 8000/mm3 with 40% lymphocytes. The concentration of serum proteins was normal. The patient was treated daily for 1 I months with a compound containing 1.5 g aceto-salisylic acid and 2.5 mg prednisolone. This treatment relieved his muscular symptoms. However, muscular stiffness in the morning persisted, his ESR remained accelerated, and he was anaemic. Discontinuance of therapy 2 weeks before admission to this hospital, in August 1971, led to a severe exacerbation of his muscular pain, further acceleration of the ESR, and a worsening of the enaemia. Examination on admission revealed painful muscles with stiffness and limitated mobility of the shoulders, as well as marked atrophy of the deltoid and humeral muscles. The sternoclavicular joints had tender swellings; radiographs of the joints revealed capsular thickening and sclerosing changes of the clavicular bone ends. Neither liver nor spleen was palpable and there was no lymphadenopathy. At the time of admission, the Hb concentration was 9.0 g/100 ml, ESR 102 mm/h, the leukocyte count 9800/mm3 with 45% lymphocytes. The concentration of serum proteins was 6.9 gll00 m], of which 1 . 1 g were alpha,globulins. The latex fixation and Waaler-Rose tests were negative. The Coombs test was positive, but there were no signs of haemolysis. In bone marrow smears, erythropoiesis and myelopoiesis were normal and lymphocytes comprised 14% of the counted cells. Results of liver enzyme tests (S-GOT, S-GPT) were normal but the alkaline phosphatase was slightly elevated and a fine-needle aspirate of the liver contained many lymphocytes, some of which were large and vacuolized and had an immature chromatin pattern. Examination of muscle and renal biopsy specimens revealed clusters of lymphocytes in the interstitium, but other tissue components appeared normal. The clinical picture was considered typical of PMR and treatment with prednisone was started. Prednisone, 25 mg per day, led to prompt relief of the patient’s muscular symptoms. H b rose to 12.5 gll00 ml, the ESR returned to normal, and the Coombs test became negative. A maintenance dose of prednisone, 5 to 10 mg a day, kept the patient free from muscular symptoms. Fig. I shows that even after the muscular symptoms had subsided, the Scand J Rheumatology 6


J . von Knorring and 0.Selroos

In the paper by von Knorring & Somer (10) we also reported on two additional patients, from another series, who had PMR and malignancy. In one of them a paraneoplastic PMR syndrome had developed that responded poorly to steroid treatment. For one year after the onset of PMR, the propositus had near-normal lymphocyte counts in both peripheral blood assays and bone marrow smears. Bone marrow 34 65 82 per cent lymphocytes l4 At the end of this year, however, lymphocytic infil1970 1971 1972 1973 1974 1975 1976 trations were observable in biopsy specimens from TAU^. S.pi.1 the liver, kidney and muscle. The lymphocytes in Fig. 1. Graphical demonstration of the clinical course. the liver aspirate were large, contained immature chromatin and thus differed morphologically from lymphocytes typically present in patients with PMR number of blood and bone marrow lymphocytes continued ( 1 1). Thereafter the increase in the number of marto increase. When, in September 1974, firm discrete lymph nodes, 1 to 1.5 cm in diameter, were palpable on his neck row and blood lymphocytes was slow but steady and in the inguinal regions the patient was readmitted to until, after 3 years, it became rapid. By that time the hospital. He had then no muscular symptoms; his liver patient had received corticosteroids continuously and spleen were not enlarged; a chest radiograph showed for 3 years which, as we suggested in our previously no hilar lymphadenopathy. Examination of the fine-needle reported case of PMR with malignant lymphoma aspirate from a lymph node, however, revealed a uniform seed of small lymphocytes with a few lymphoblasts, a (8), might have retarded the development of CLL. finding typical of CLL. Study of the bone marrow showed It must be borne in mind, however, that the natural it to be heavily infiltrated with lymphocytes. Neither T- course of CLL is slow. nor B-lymphocytes were detected in peripheral blood, but As for the lymphocyte subpopulations in the proabout 95% of the blood lymphocytes bore Fc receptors. positus, neither T- nor B-lymphocytes could be Because the number of blood and bone marrow lymdemonstrated in the blood with conventional cellphocytes increased rapidly, a regimen of chlorambucil 10 mg per day and prednisone 10 mg per day was started. surface markers. Hence, 95% of the lymphocytes Response to this treatment has been good. At the most were Fc receptor-bearing cells whose origin is unrecent follow-up examination, in September 1976, the paknown. The Fc receptor has been observed on vartient had no muscular symptoms and no lymphadenopathy. His ESR was 12 mm/h, Hb 10.8 g/lOO ml, and the ious types of cells, including activated T cells, and in a variety of malignant cell populations, e.g. leukocyte count was 58 000/mm3 with 95% lymphocytes. malignant lymphomas, plasma-cell tumours or, as in this case, lymphatic leukaemia (7). DISCUSSION The case of the propositus resembles that of a During the follow-up period, 1967-75, 4 of our patient reported by Jakobson (6). Marked lymphoseries of 51 patients with PMR or TA, have de- cytosis was already noted at the onset of acute TA veloped a malignant disease, including the proposi- in his patient, but it subsided along with the symptus. In the first patient a malignant lymphoma was toms of TA over a 4-month course with corticostediagnosed 4 years after the onset of PMR (8), and he roids. Whether the lymphocytic reaction was secsubsequently died of a pulmonary embolism. The ondary to the TA, or whether it heralded the onset second patient died of a metastasizing adenocarci- of CLL, was therefore unclear. In our patient the noma of unknown origin 3 years after TA had been association of CLL with PMR may simply be diagnosed (10). The third patient was treated surgi- coincidental coexistence of two unrelated diseases, cally for a non-metastasizing breast carcinoma 3 both relatively common in elderly persons. Howyears after the onset of PMR (9). Inconsistent with a ever, the clinical course does point to a more direct paraneoplastic mechanism of the rheumatoid symp- relationship. In previously reported cases of toms in all three cases were the long interval be- malignancy in the presence of PMR, the malignant tween the onset of PMR or TA and the diagnosis of tumour has been detected 2 4 years after onset of malignancy; the absence of active muscular symp- symptoms due to PMR or TA (1, 3, 4, 14). In our toms when the neoplasm was detected; and the patient, as well as in the patient reported by excellent response to corticosteroid treatment (10). Jakobson (6), infiltration of abnormal lymphocytes Chlorernbucll(mg)



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Scund J Rheurnutology 6

Polyrnyalgia rheurnatica and chronic lymphatic leukaernia

was documented at an early stage of the rheumatic disease. It may be that in these patients the PMR syndrome does indeed represent a paraneoplastic immunophenomenon that results from a slowly progressing CLL. In view of this possibility, a CLL in progress must be suspected when persistent lymphocytosis occurs in a patient with typical PMR or TA, even if the patient’s primary response to corticosteroid therapy is good. ACKNOWLEDGEMENT The authors are grateful to Tom Pettersson, M.D., for performing the determination of the lymphocyte subpopulations. For technical references see (13).

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REFERENCES I . Andrews, F. M.: Polymyalgia rheurnatica. A biopsy and follow-up study. Ann Rheum Dis24:432, 1965. 2. Cammarata, R. J., Rodman, G. P. & Jensen, N. N.: Systemic rheumatic disease and malignant lymphoma. Arch Intern Med 111: 330, 1%3. 3. Hamrin, B., Jonsson, N. & Hellsten, S.: “Polymyalgia rheumatica.” Further clinical and histopathological studies with a report of six autopsy cases. Ann Rheum Dis 27: 397, 1%8. 4. Hauser, W. A., Ferguson, R., Holley, K. & Kurland, L.: Temporal arteritis in Rochester, Minnesota, 1951 to 1%7. Mayo Clin Proc46:597, 1971. 5 . Hench, P. K., Mayne, J. G., Kiely, J. M. & Dockerty, M.B.: Clinical study of the rheumatic manifestations of lymphoma. Abstract Arthr Rheum 5: 301, 1%2.


6. Jakobson, T.: Lymfatisk reaktion vid arteritis temporalis. Nord Med 50: 1741, 1953. 7. Kerbel, R. S. & Davies, A. J. S.: The possible biological significance of Fc receptor on mammalian lymphocytes and tumor cells. Cell3: 105, 1974. 8. v. Knomng, J.: Polymyalgia rheumatica and malignant lymphoma. Scand J Rheumatology I: 117, 1972. 9. v. Knomng, J. & Selroos, 0.:Sarcoidosis with thyroid involvement, polymyalgia rheumatica and breast carcinoma. Scand J Rheumatology 5: 77, 1976. 10. v. Knomng, J. & Somer, T.: Malignancy in association with polymyalgia rheumatica and temporal arteritis. Scand J Rheumatology3: 129, 1974. 1 I . v. Knomng, J. & Wasastjerna, C.: Liver involvement in polymyaba rheumatica. Scand J Rheumatology 5 : 197, 1976. 12. Lea, A. J.: An association between the rheumatica diseases and the reticuloses. Ann Rheum Dis 23:480, 1964.

13. Wangel, A. & Klockars, M.: Lymphocyte subpopulations in rheumatoid synovial tissue. Ann Rheum Dis, 1976 (in press). 14. Ostberg, G.: Morphological changes in the large arteries in polymyalgia arteritica. Acta Med Scand, Suppl. 533: 135, 1972. Submitted for publication October 28, 1976

Johan von Knomng Fourth Department of Medicine Helsinki University Central Hospital Unionsgatan 38 SF-00170 Helsinki 17 Finland

Scand J Rheumatology 6

Polymyalgia rheumatica and chronic lymphatic leukaemia.

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