Linfadenopatía Angioinmunoblástica con disproteinemia en una mujer de 23 años: Linfoma de células T periférico
Palabras clave:
linfadenopatía angioinmunoblástica, hipergamaglobulinemia policlonal, linfoma de células T periféricoResumen
La linfadenopatía angioinmunoblástica es un linfoma de células T periférico, caracterizado por síntomas constitucionales, como pérdida de peso, fiebre, linfadenopatías y hepatoesplenomegalia; igualmente signos cutáneos inespecíficos, como eritema máculo-papular y eritrodermia, que se acompañan de leucocitosis, eosinofilia, velocidad de sedimentación aumentada e hipergamaglobulinemia policlonal. El rearreglo clonal del gen del receptor de las células T y la histología dada por distorsión de la arquitectura ganglionar, un infitrado lintehistiocitario con inmunoblastos e hiperplasia vascular, confirman el diagnóstico.
Presentamos el caso de una mujer joven, con prurito crónico, pigmentación grisácea generalizada, fiebre, pérdida de peso y adenopatías con linfadenopatía angioinmunoblástica. E.1 diagnóstico se configuró con los hallazgos clínicos e histológicos ganglionares típicos. La paciente inició tratamiento con poliquimioterapia respondiendo adecuadamente; sin embargo, un mes después de terminar los ciclos, fue nuevamente hospitalizada por un cuadro neumónico que la llevó a la muerte.
Biografía del autor/a
María Victoria Suárez, Universidad Nacional
Residente Dermatología Universidad Nacional, Santafé de Bogotá.
Referencias bibliográficas
1. Steinberg AD, Seldin MF, Jaffe ES, et al. Angioimmunoblasticlymphadenopathywith disproteinemia.Ann lnt Med 1988; 108: 575-584.
https://doi.org/10.7326/0003-4819-108-4-575
2. Seehafer JR, Goldberg NC, Dicken CH et al. Cutaneous manifestations of angioimmunoblastic lymphadenopathy. Arch Dermatol 1980; 116:41-45. Schmuth M, Ramaker J, Trautmann C, et al. Cuta- neous involvement in prelymphomatous angioimmu- noblastic lymphadenopathy. J Am Acad Dermatol 1997; 36:290-295.
https://doi.org/10.1016/S0190-9622(97)80401-X
4. Bernengo MG, Levi L, Zina G, et al. Skin lesions in angioimmunoblastic lymphadenopathy: histological and immunological studies. Br J Dermatol 1981; 104: 131-139.
https://doi.org/10.1111/j.1365-2133.1981.tb00034.x
5. Knecht H. Angioimmunoblastic lymphadenopathy: Ten years, experience and state of current knowledge. Seminars in Hematology 1989; 26:208-215.
6. Frizzera G, Moran EM, Rappaport H. Angio-immunoblastic lymphadenopathy with disproteinemia. Lancet 1974; 1:1070-1073.
https://doi.org/10.1016/S0140-6736(74)90553-4
Lukes RJ, Tindle BH. lmmunoblastic lymphadenopathy: a hiperimmune entity resembling Hodgkin's disease. N Engl J Med 1975; 292:1-8.
https://doi.org/10.1056/NEJM197501022920101
8. Radaszkiewicz T, Lennert K. Lymphogranulomatosis X. Klinisches Bild. Therapies and prognose. Dtsch Med Wosenschr. 1975; 100:1157-1163.
https://doi.org/10.1055/s-0028-1106350
9. Harris NL, Jaffe ES, Stein H, et al. A revised European- American classification of lymphoid neoplasm: a proposal from the lnternational Lymphoma Study Group. Blood 1994; 84:1361-1392
10. Fiorillo A, Pettinato G, Raia V, et al. Angioimmunoblastic lymphadenopathy with disproteinemia: report of the first case in chilhood evolving toward sponta- neous remission. Cancer 1981; 48: 1611-1614.
https://doi.org/10.1002/1097-0142(19811001)48:7<1611::AID-CNCR2820480723>3.0.CO;2-X
11. Nakano A, Hatta K, Kbashi Y, et al. Salazosulfapyridine-induced angioimmunoblastic lymphadenopathy. lnter Med 1996; 35: 894-897.
https://doi.org/10.2169/internalmedicine.35.894
12. Luppi M, Torelli G. The new lymphotropic herpes viruses (HHV-6' HHV-7' HHV-8) in human lympho-proliferative diseases: an overview. Haematologica 1996; 81: 265-281.
13. Schuster V, Ott G, Seidenspinner S et al. Common Epstein-Barr virus (EBV) type 1 variant strains in both malignant and benign EBV-associated disorders. Blood 1996; 87: 1579-1585.
14. Azevedo SJ, Yunis AA. Angioimmunoblastic lymphadenopathy, Am J Hematol 1985; 20: 301-312.
https://doi.org/10.1002/ajh.2830200314
15. Siegert W, Agthe A, Greisser H, et al. Treatment of angioimmunoblastic lymphadenopathy (AILD) - type T-cell lymphoma using prednisone with or without the COPBLAM/IMVP- 16 regimen. Ann lntern Med 1992; 117: 364-370.
https://doi.org/10.7326/0003-4819-117-5-364
16. Paloczi K, Surayani P, Nemes Z, et al. A study of lymphocyte subsets in patients with angioimmu noblastic lymphadenopathy. Br J Haematol 1986; 62: 615-618.
https://doi.org/10.1111/j.1365-2141.1986.tb04083.x
17. Honda M, Smith HR, Steinberg AD. Studies of the pathogenesis of angioimmunoblastic lymphadeno pathy. J Clin lnvest 1985; 76: 332-340.
https://doi.org/10.1172/JCI111966
18. Foss HD, Anagnotopoulos 1, Hummel M, et al. Patterns of cytokine gene expression in peripheral T- cell lymphoma of angioimmunoblastic lympha denopathy type. Blood 1995; 85:2862-2869.
19. Greisser H, Feller AC, Lennert K, et al. Rearran gement of beta chain of the T-cell antigen receptor and immunoglobulin genes in lymphoproliferative disorders. J Clin lnvest 1986; 78:1179-1184.
https://doi.org/10.1172/JCI112700
20. Greisser H, Tkachuk D, Reis MD, et al. Gene rearran gements and translocations in lymphoproliferative disorders. Blood 1989; 73:1402-1415.
21. Godde-Salz E, Feller A, Lennert K, et al. Chromo somal abnormalities in lymphogranulomatosis X (LgrX)/ angioimmunoblastic lymphadenopathy (AILD). Leuk Res 1987; 11:181-190.
https://doi.org/10.1016/0145-2126(87)90024-5
22. Klinman DM, Steinberg AD, Mushinski JF. Effect of cyclophosphamide therapy on oncogene expression in angioimmunoblastic lymphadenopathy. Lancet 1986; 11:1055-1058.
https://doi.org/10.1016/S0140-6736(86)90464-2
23. Anagnotopoulos 1, Hummel M, Finn T, et al. Hetero geneous Epstein-Barr virus infection patterns in peripheralT-celllymphoma ofangioimmunoblastic lymphadenopathy type. Blood 1992; 80:1804.
24. Abruzzo LV, Schmidt K, Weiss LM, et al. 8-cell lymphoma after angioimmunoblastic lymphadenopathy; a case with oligoclonal gene rearrangements associated with Epstein-Barr virus. Blood 1993; 82: 241.
25. Seigert F, Schwwerdtfeger R, Hartmann R, et al. LymphogranulomatosisX. Amulticenterevaluation of a standardized treatment. Blood 1989; 74: 280.
https://doi.org/10.7326/0003-4819-108-4-575
2. Seehafer JR, Goldberg NC, Dicken CH et al. Cutaneous manifestations of angioimmunoblastic lymphadenopathy. Arch Dermatol 1980; 116:41-45. Schmuth M, Ramaker J, Trautmann C, et al. Cuta- neous involvement in prelymphomatous angioimmu- noblastic lymphadenopathy. J Am Acad Dermatol 1997; 36:290-295.
https://doi.org/10.1016/S0190-9622(97)80401-X
4. Bernengo MG, Levi L, Zina G, et al. Skin lesions in angioimmunoblastic lymphadenopathy: histological and immunological studies. Br J Dermatol 1981; 104: 131-139.
https://doi.org/10.1111/j.1365-2133.1981.tb00034.x
5. Knecht H. Angioimmunoblastic lymphadenopathy: Ten years, experience and state of current knowledge. Seminars in Hematology 1989; 26:208-215.
6. Frizzera G, Moran EM, Rappaport H. Angio-immunoblastic lymphadenopathy with disproteinemia. Lancet 1974; 1:1070-1073.
https://doi.org/10.1016/S0140-6736(74)90553-4
Lukes RJ, Tindle BH. lmmunoblastic lymphadenopathy: a hiperimmune entity resembling Hodgkin's disease. N Engl J Med 1975; 292:1-8.
https://doi.org/10.1056/NEJM197501022920101
8. Radaszkiewicz T, Lennert K. Lymphogranulomatosis X. Klinisches Bild. Therapies and prognose. Dtsch Med Wosenschr. 1975; 100:1157-1163.
https://doi.org/10.1055/s-0028-1106350
9. Harris NL, Jaffe ES, Stein H, et al. A revised European- American classification of lymphoid neoplasm: a proposal from the lnternational Lymphoma Study Group. Blood 1994; 84:1361-1392
10. Fiorillo A, Pettinato G, Raia V, et al. Angioimmunoblastic lymphadenopathy with disproteinemia: report of the first case in chilhood evolving toward sponta- neous remission. Cancer 1981; 48: 1611-1614.
https://doi.org/10.1002/1097-0142(19811001)48:7<1611::AID-CNCR2820480723>3.0.CO;2-X
11. Nakano A, Hatta K, Kbashi Y, et al. Salazosulfapyridine-induced angioimmunoblastic lymphadenopathy. lnter Med 1996; 35: 894-897.
https://doi.org/10.2169/internalmedicine.35.894
12. Luppi M, Torelli G. The new lymphotropic herpes viruses (HHV-6' HHV-7' HHV-8) in human lympho-proliferative diseases: an overview. Haematologica 1996; 81: 265-281.
13. Schuster V, Ott G, Seidenspinner S et al. Common Epstein-Barr virus (EBV) type 1 variant strains in both malignant and benign EBV-associated disorders. Blood 1996; 87: 1579-1585.
14. Azevedo SJ, Yunis AA. Angioimmunoblastic lymphadenopathy, Am J Hematol 1985; 20: 301-312.
https://doi.org/10.1002/ajh.2830200314
15. Siegert W, Agthe A, Greisser H, et al. Treatment of angioimmunoblastic lymphadenopathy (AILD) - type T-cell lymphoma using prednisone with or without the COPBLAM/IMVP- 16 regimen. Ann lntern Med 1992; 117: 364-370.
https://doi.org/10.7326/0003-4819-117-5-364
16. Paloczi K, Surayani P, Nemes Z, et al. A study of lymphocyte subsets in patients with angioimmu noblastic lymphadenopathy. Br J Haematol 1986; 62: 615-618.
https://doi.org/10.1111/j.1365-2141.1986.tb04083.x
17. Honda M, Smith HR, Steinberg AD. Studies of the pathogenesis of angioimmunoblastic lymphadeno pathy. J Clin lnvest 1985; 76: 332-340.
https://doi.org/10.1172/JCI111966
18. Foss HD, Anagnotopoulos 1, Hummel M, et al. Patterns of cytokine gene expression in peripheral T- cell lymphoma of angioimmunoblastic lympha denopathy type. Blood 1995; 85:2862-2869.
19. Greisser H, Feller AC, Lennert K, et al. Rearran gement of beta chain of the T-cell antigen receptor and immunoglobulin genes in lymphoproliferative disorders. J Clin lnvest 1986; 78:1179-1184.
https://doi.org/10.1172/JCI112700
20. Greisser H, Tkachuk D, Reis MD, et al. Gene rearran gements and translocations in lymphoproliferative disorders. Blood 1989; 73:1402-1415.
21. Godde-Salz E, Feller A, Lennert K, et al. Chromo somal abnormalities in lymphogranulomatosis X (LgrX)/ angioimmunoblastic lymphadenopathy (AILD). Leuk Res 1987; 11:181-190.
https://doi.org/10.1016/0145-2126(87)90024-5
22. Klinman DM, Steinberg AD, Mushinski JF. Effect of cyclophosphamide therapy on oncogene expression in angioimmunoblastic lymphadenopathy. Lancet 1986; 11:1055-1058.
https://doi.org/10.1016/S0140-6736(86)90464-2
23. Anagnotopoulos 1, Hummel M, Finn T, et al. Hetero geneous Epstein-Barr virus infection patterns in peripheralT-celllymphoma ofangioimmunoblastic lymphadenopathy type. Blood 1992; 80:1804.
24. Abruzzo LV, Schmidt K, Weiss LM, et al. 8-cell lymphoma after angioimmunoblastic lymphadenopathy; a case with oligoclonal gene rearrangements associated with Epstein-Barr virus. Blood 1993; 82: 241.
25. Seigert F, Schwwerdtfeger R, Hartmann R, et al. LymphogranulomatosisX. Amulticenterevaluation of a standardized treatment. Blood 1989; 74: 280.
Cómo citar
1.
Suárez MV. Linfadenopatía Angioinmunoblástica con disproteinemia en una mujer de 23 años: Linfoma de células T periférico. rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 1 de febrero de 2000 [citado 20 de abril de 2024];8(1):71-6. Disponible en: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/647
Descargas
Los datos de descargas todavía no están disponibles.
Descargas
Publicado
2000-02-01
Cómo citar
1.
Suárez MV. Linfadenopatía Angioinmunoblástica con disproteinemia en una mujer de 23 años: Linfoma de células T periférico. rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 1 de febrero de 2000 [citado 20 de abril de 2024];8(1):71-6. Disponible en: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/647
Número
Sección
Casos Clínicos
| Estadísticas de artículo | |
|---|---|
| Vistas de resúmenes | |
| Vistas de PDF | |
| Descargas de PDF | |
| Vistas de HTML | |
| Otras vistas | |
