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Immunoblastic Lymphadenopathy With Purpura and Cryoglobulinemia

Immunoblastic Lymphadenopathy With Purpura and Cryoglobulinemia Abstract • Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan. (Arch Dermatol 113:636-641, 1977) References 1. Frizzera G, Moran EM, Rappaport H: Angioimmunoblastic lymphadenopathy with dysproteinemia . Lancet 1:1070-1073, 1974.Crossref 2. Lukes RJ, Tindle BH: Immunoblastic lymphadenopathy: A hyperimmune entity resembling Hodgkin's disease . N Engl J Med 292:1-8, 1975.Crossref 3. Radaszkiewicz T, Lennert K: Lymphogranulomatis X: Klinishes, bild therapie and prognose . Dtsch Med Wochenschr 100:1157-1163, 1975.Crossref 4. Schultz DR, Yunis AA: Immunoblastic lymphadenopathy with mixed cryoglobulinemia: A detailed case study . N Engl J Med 292:8-12, 1975.Crossref 5. Rappaport H, Moran EM: Angio-immunoblastic (immunblastic) lymphadenopathy, editorial . N Engl J Med 292:42-43, 1975.Crossref 6. Twomey JJ: Angio-immunoblastic lymphadenopathy with dysproteinemia, letter to the editor . Lancet 1:1345, 1974.Crossref 7. Horne CHW, Fraser RA, Petrie JC: Angioimmunoblastic lymphadenopathy with dysproteinemia, letter to the editor . Lancet 2:291, 1974.Crossref 8. Cormane RH, Szabo E, Hauge LS: Immunofluorescence of the skin: The interpretation of the staining of blood vessels and connective tissue aided by new techniques . Br J Dermatol 82 ( (suppl 5) ):26-43, 1970. 9. Fahey JL, McKelvey EM: Quantitative determination of serum immunoglobulins in antibody-agar plates . J Immunol 94:84-90, 1965. 10. Schriedegger JJ: Une micro-methode de immuno-electrophorése . Int Arch Allergy Appl Immunol 7:103-110, 1955.Crossref 11. Nakamura RM: Immunopathology, Clinical Laboratory Concepts and Methods . Boston, Little Brown & Co, 1974, pp 190-191. 12. Böyum A: Isolation of leucocytes from human blood: Further observations . Scan J Clin Lad Invest 21( (suppl 97) ):31-50, 1968.Crossref 13. Jondal M, Holm G, Wigzell H: Surface markers on human T and B lymphocytes: I. A large population of lymphocytes forming nonimmune rosettes with sheep red blood cells . J Exp Med 136:207-215, 1972.Crossref 14. Grey HM, Rabellino E, Pirofsky B: Immunoglobulins of the surface of lymphocytes . J Clin Invest 50:2368-2375, 1971.Crossref 15. Junge U, Hoekstra J, Wolfe L, Deinhardt F: Microtechnique for quantitative evaluation of in vitro lymphocyte transformation . Clin Exp Immunol 7:431-437, 1970. 16. Cream JJ: Immunofluorescent studies of the skin in cryoglobulinemic vasculitis . Br J Dermatol 84:48-53, 1971.Crossref 17. Brouet J-C, Clauvel J-P, Danon F, et al: Biologic and clinical significance of cryoglobulins: A report of 86 cases . Am J Med 57:775-788, 1974.Crossref 18. Shuster J: Disorders associated with hypergammaglobulinemia and proteinemia , in Friedman SO (ed): Clinical Immunology . New York, Harper & Row Publishers Inc, 1971, chapt 10, pp 314-341. 19. Yu DTY, Clements PJ, Paulus HE, et al: Human lymphocyte subpopulations effect of corticosteroids . J Clin Invest 53:565-571, 1974.Crossref 20. Jeter WS, Seebom PM: Effect of cortisone and adrenocorticotropic hormone of delayed hypersensitivity to 2,4-dinitrocholorbenzene in guinea pigs . J Immunol 73:44-48, 1954. 21. Bovornkitti S, Kangsadal P, Sathirapat P, et al: Reversion and reconversion rate of tuberculin skin reactions in correlation with the use of prednisone . Dis Chest 38:51-55, 1960.Crossref 22. Claman HN: Medical progress: Corticosteroid and lymphoid cells . N Engl J Med 287:388-397, 1972.Crossref 23. Salzstern SL, Ackerman LV: Lymphadenopathy induced by anticonvulsant drugs and mimicking clinically and pathologically malignant lymphoma . Cancer 12:164-182, 1959.Crossref 24. Schwartz RS, Beldotti L: Malignant lymphomas following allogenic disease: Transition from an immunological to a neoplastic disease . Science 140:1511-1514, 1965.Crossref 25. Anderson LG, Talal N: The spectrum of benign to malignant lymphoproliferation in Sjogren's syndrome . Clin Ext Immunol 9:199-221, 1971. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Immunoblastic Lymphadenopathy With Purpura and Cryoglobulinemia

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Publisher
American Medical Association
Copyright
Copyright © 1977 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1977.01640050096015
Publisher site
See Article on Publisher Site

Abstract

Abstract • Immunoblastic lymphadenopathy is a recently described lymphoproliferative disorder, presumably of B-cell origin. It is characterized by regional or generalized lymphadenopathy, usually associated with hypergammaglobulinemia or dysproteinemia. Other findings may be hepatosplenomegaly, dermatitis, fever, malaise, weight loss, and various altered immunologic reactions. Histologically, the involved lymph nodes show immunoblast, plasmacytoid, and plasma cell proliferation. This may be extranodal as well. The case reported here is one of the few followed up prospectively. The patient's purpuric eruption was an apparent manifestation of a type II mixed cryoglobulinemia. Differing from what has usually been reported, we noted hypogammaglobulinemia and findings in part of altered cell-mediated immunity. Despite leukopenia and anemia there were no infectious episodes. Although a satisfactory treatment regimen has not been established, there was beneficial response to prednisone and short courses of melphalan. (Arch Dermatol 113:636-641, 1977) References 1. Frizzera G, Moran EM, Rappaport H: Angioimmunoblastic lymphadenopathy with dysproteinemia . Lancet 1:1070-1073, 1974.Crossref 2. Lukes RJ, Tindle BH: Immunoblastic lymphadenopathy: A hyperimmune entity resembling Hodgkin's disease . N Engl J Med 292:1-8, 1975.Crossref 3. Radaszkiewicz T, Lennert K: Lymphogranulomatis X: Klinishes, bild therapie and prognose . Dtsch Med Wochenschr 100:1157-1163, 1975.Crossref 4. Schultz DR, Yunis AA: Immunoblastic lymphadenopathy with mixed cryoglobulinemia: A detailed case study . N Engl J Med 292:8-12, 1975.Crossref 5. Rappaport H, Moran EM: Angio-immunoblastic (immunblastic) lymphadenopathy, editorial . N Engl J Med 292:42-43, 1975.Crossref 6. Twomey JJ: Angio-immunoblastic lymphadenopathy with dysproteinemia, letter to the editor . Lancet 1:1345, 1974.Crossref 7. Horne CHW, Fraser RA, Petrie JC: Angioimmunoblastic lymphadenopathy with dysproteinemia, letter to the editor . Lancet 2:291, 1974.Crossref 8. Cormane RH, Szabo E, Hauge LS: Immunofluorescence of the skin: The interpretation of the staining of blood vessels and connective tissue aided by new techniques . Br J Dermatol 82 ( (suppl 5) ):26-43, 1970. 9. Fahey JL, McKelvey EM: Quantitative determination of serum immunoglobulins in antibody-agar plates . J Immunol 94:84-90, 1965. 10. Schriedegger JJ: Une micro-methode de immuno-electrophorése . Int Arch Allergy Appl Immunol 7:103-110, 1955.Crossref 11. Nakamura RM: Immunopathology, Clinical Laboratory Concepts and Methods . Boston, Little Brown & Co, 1974, pp 190-191. 12. Böyum A: Isolation of leucocytes from human blood: Further observations . Scan J Clin Lad Invest 21( (suppl 97) ):31-50, 1968.Crossref 13. Jondal M, Holm G, Wigzell H: Surface markers on human T and B lymphocytes: I. A large population of lymphocytes forming nonimmune rosettes with sheep red blood cells . J Exp Med 136:207-215, 1972.Crossref 14. Grey HM, Rabellino E, Pirofsky B: Immunoglobulins of the surface of lymphocytes . J Clin Invest 50:2368-2375, 1971.Crossref 15. Junge U, Hoekstra J, Wolfe L, Deinhardt F: Microtechnique for quantitative evaluation of in vitro lymphocyte transformation . Clin Exp Immunol 7:431-437, 1970. 16. Cream JJ: Immunofluorescent studies of the skin in cryoglobulinemic vasculitis . Br J Dermatol 84:48-53, 1971.Crossref 17. Brouet J-C, Clauvel J-P, Danon F, et al: Biologic and clinical significance of cryoglobulins: A report of 86 cases . Am J Med 57:775-788, 1974.Crossref 18. Shuster J: Disorders associated with hypergammaglobulinemia and proteinemia , in Friedman SO (ed): Clinical Immunology . New York, Harper & Row Publishers Inc, 1971, chapt 10, pp 314-341. 19. Yu DTY, Clements PJ, Paulus HE, et al: Human lymphocyte subpopulations effect of corticosteroids . J Clin Invest 53:565-571, 1974.Crossref 20. Jeter WS, Seebom PM: Effect of cortisone and adrenocorticotropic hormone of delayed hypersensitivity to 2,4-dinitrocholorbenzene in guinea pigs . J Immunol 73:44-48, 1954. 21. Bovornkitti S, Kangsadal P, Sathirapat P, et al: Reversion and reconversion rate of tuberculin skin reactions in correlation with the use of prednisone . Dis Chest 38:51-55, 1960.Crossref 22. Claman HN: Medical progress: Corticosteroid and lymphoid cells . N Engl J Med 287:388-397, 1972.Crossref 23. Salzstern SL, Ackerman LV: Lymphadenopathy induced by anticonvulsant drugs and mimicking clinically and pathologically malignant lymphoma . Cancer 12:164-182, 1959.Crossref 24. Schwartz RS, Beldotti L: Malignant lymphomas following allogenic disease: Transition from an immunological to a neoplastic disease . Science 140:1511-1514, 1965.Crossref 25. Anderson LG, Talal N: The spectrum of benign to malignant lymphoproliferation in Sjogren's syndrome . Clin Ext Immunol 9:199-221, 1971.

Journal

Archives of DermatologyAmerican Medical Association

Published: May 1, 1977

References

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