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| Year : 1982 | Volume
: 28
| Issue : 4 | Page : 238-40 |
Waldenström's macroglobulinemia. (A case report).
Vakil NB, Agarwal MB, Tilve GH
How to cite this article:
Vakil N B, Agarwal M B, Tilve G H. Waldenström's macroglobulinemia. (A case report). J Postgrad Med 1982;28:238 |
Macroglobulinemia was first described in 1944 by Waldenstrom.[11] It is a neoplastic proliferation of plasma cells and B lymphocytes that are normally responsible for the synthesis of the heavy chains. In contrast to lymphatic leukemia, they maintain a capacity to differentiate and mature into plasma cells. The cells in Waldenstrom's macroglobulinemia, therefore, have features which range from lymphoid to mature plasma cells. Immunofluorescence studies of cells obtained from bone marrow, lymph nodes and blood of patients with this disease show detectable cytoplasmic IgM in plasma cells and surface immunoglobulin on the vast majority of lymphocytes.[10]
M.C., a 62 year old male developed anorexia and general debility 6 months before admission. One week before admission, he developed a purpuric rash over both the lower limbs and buttocks.
On examination, he was found to be of medium build and poorly nourished. There was mild pallor and purpura over both the lower limbs and buttocks and a few purpuric spots on the trunk. The upper limbs and the face were unaffected. The liver was enlarged (6 cm), firm, smooth and not-tender. The spleen was also enlarged (10 cm).
Laboratory investigations revealed haemoglobin of 9.7 gm%. Haematocrit was 29% MCHC 34%, Retic count 1.9% WBC 14,900/cmm; with neutrophils 85% and lymphocytes 15%; Platelet count was normal. There were no abnormal cells. Urine examination showed mild albuminuria (5-6 RBC/high power field). Urinary Bence Jones proteins were positive. The blood urea nitrogen was 48 mg% with a serum creatinine of 3.5 mg%. Liver function tests were within normal limits. An X-ray of the chest revealed enlarged paratracheal lymph nodes. A radionucleide bone scan was normal. Bone marrow aspiration biopsy was normal. A bone marrow trephine biopsy showed 5% plasma cells. Total serum proteins were 10.6 gm% and electrophoresis showed IgM to be elevated to 3.5 g m%.
The average age of diagnosis of Waldenstrom's macroglobulinemia is 60 years and almost two thirds of the patients are males. Most patients are detected when they develop symptoms, the commonest of which are fatigue, weakness and bleeding (epistaxis, purpura) which are presenting symptoms in half the patients. Weight loss, visual disturbances, Raynaud's phenomenon are the less common symptoms. The usual physical signs are hepatosplenomegaly and lymphadenopathy. Dilated tortuous retinal veins may be present. Purpura and neurological abnormalities consisting of confusion and altered conciousness, called coma paraproteinaemicum, also occur.
The association of macroglobulinemia with neurological symptoms has been referred to as the Bing-Neel syndrome.[1] Polyneuropathy and cardiac failure also occur in some patients.[3], [4]
A normochromic normocytic anaemia is usually present.[2] Leucocytosis, thrombocytopenia and osteolytic bone lesions are rare. The Sia test is a simple screening test to recognise the presence of euglobulins. A drop of serum is added to a cylinder of distilled water and if a filmy white precipitate, appears as the drop settles, the test is read as positive. The majority of patients with Waldenstrom's macroglobulinemia are Sia positive but a negative test does not rule out the diagnosis.[6]
Bence Jone's proteinuria is present in one fourth of all patients with Waldenstrom's macroglobulinemia and is usually due to a predominantly glomerular lesion with deposits of IgM and amyloid like material.[9] An immunologically mediated renal disease has also been described.[8] Platelet function is impaired by the coating of platelets with IgM and some patients show coagulation defects which may be due to the interaction of the IgM protein with labile coagulation factors. IgM levels are markedly raised and cryoglobulin may be detected is some patients. The clinical manifestations of macroglobulinemia may resemble those of chronic lymphatic leukemia, lymphoma or the plasma cell neoplasia. The demonstration of a serum component containing the M heavy chains is diagnostic of macroglobulinemia. As this is a rare disease with a variable course, the effectiveness of antineoplastic therapy is not established. In one study[5] of 31 patients, a beneficial effect with chlorambucil was found. In another study[7] of 40 patients, 8 out of 19 patients treated with chlorambucil and cyclophosphamide or melphalan showed a fall in IgM levels. The average survival time was 49.2 months for the responders and only 24.1 months for non-responders. The optimum dosage and treatment schedule are as yet undetermined. The disease usually progresses gradually over several years. Hyperviscosity, haemorrhage, thrombotic episodes and infections are the usual causes of death.
| 1. | Bichel, J., Bing, J. and Harboe, N.: Another case of hyperglobulinemia and affection of the central nervous system. Acts. Med. Scand., 138: 1-14, 1950.  |
| 2. | Cline, M, J., Solomen, A., Berlin, N. L. and Fahey, J. L.: Anemia in macroglobulinemia. Amer. J. Med., 34: 213-220, 1963. |
| 3. | Clinocopathological Conference: Macroglobulinemia. Amer. J. Med., 28: 951-962, 1960. |
| 4. | Darnley, J. D.: Polyneuropathy in Waldenstrom's macroglobulinemia. A case report and discussion. Neurology, 12: 617-623, 1962. |
| 5. | McCallister, B. D., Bayrd, E. D., Harrison, E. G. Jr. and McGuckin, W. F.: Review: Primary macroglobulinemia. Review with a report on 31 cases and notes on the value of continuous chlorambucil therapy. Amer. J. Med., 43: 394-434, 1967. |
| 6. | MacKay, I. R., Eriksen, N., Motulsky, A. G. and Volwiler, W.: Cryo- and macroglobulinemia. Electrophoretic, ultracentrifugal and clinical studies. Amer. J. Med., 20: 564-587, 1956. |
| 7. | MacKenzie, M. R. and Fudenberg, H. H.: Macroglobulinemia: An analysis of forty patients. Blood, 39:874-8189, 1972. |
| 8. | Martelo, O. J., Schultz, D. R.. Pardo, V. and Perez-Stable, E.: Immunologically mediated renal disease in Waldenstrom's macroglobulinemia. Amer. J. Med., 58: 567-575, 1975. |
| 9. | Morel-Maroger, L., Basch, A., Danon, F., Verroust, P. and Richet, G.: Pathology of the kidney in Waldenstrom's macroglobulinemia. Study of 16 cases. New Engl. J. Med., 283: 123-129, 1970. |
| 10. | Preud'homme, J. and Seligmann, M.: Immunoglobulins on the surface of the lymphoid cells in Waldenstrom's macroglobulinemia. J. Clin. Invest., 51: 701705, 1972. |
| 11. | Waldenstrom, J.: Incipient myelomatosis or "essential" hyperglobulinemia with fibrinogenopenia. New syndrome? Acta Med. Scand., 117: 216-247, 1941. |
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