eMedicine Specialties > Dermatology > Lymphoma and Related Processes

Angioimmunoblastic Lymphadenopathy With Dysproteinemia: Treatment & Medication

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Contributor Information and Disclosures

Updated: Feb 11, 2008

Treatment

Medical Care

Polychemotherapy is often used. Prednisone is a first-line agent in the treatment of AILD. Regimens include prednisone alone or cyclophosphamide and prednisone, cyclophosphamide with vincristine, and prednisone. Cyclophosphamide, hydroxydaunorubicin, Oncovin (vincristine), and prednisone (CHOP)–like regimens used as first-line therapy before or after steroids administration, with or without interferon alfa as consolidation, in retrospective analyses have produced complete remission rates of about 60%.

Two thirds of patients treated with low doses of recombinant interferon alfa-2a (used as a single agent) achieved an objective remission, while, in the remaining one third of patients, no change or progressive disease was observed. The median remission duration was 3.5 months. Thus, interferon seems a promising agent in the treatment of AILD, but its role must be further defined.

Radiation therapy has been used as well. Cyclosporine and 2-chlorodeoxyadenosine and cyclophosphamide have also been used. The treatment regimen of cyclophosphamide, Oncovin (vincristine), prednisone, bleomycin, Adriamycin (doxorubicin), and Matulane (procarbazine) (COP-BLAM) and ifosfamide, mesna uroprotection, methotrexate, and etoposide (IMVP-16) has been used. Methotrexate has not been found to be effective.

High-dose chemotherapy (HDCT) followed by autologous bone marrow transplantation (ABMT) represents a promising new treatment modality for patients with advanced lymphoma, conceivably including AILD.

A 5-month-old girl with AILD was treated with interferon alfa, cyclosporine A, deoxyspagarine, and azathioprine, alternating regimens of low-dose 6-mercaptopurine, cyclophosphamide, and methotrexate, and results were inconsistent. At age 58 months, a splenectomy was performed, which induced a prolonged complete remission of the AILD, without any medication.

Halene et al21 reported sustained remission from angioimmunoblastic T-cell lymphoma induced by alemtuzumab.

Rodríguez et al22 described prolonged survival for patients with angioimmunoblastic T-cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation.

Dunleavy et al3 have suggested that novel therapeutic strategies including immunomodulation with agents such as cyclosporine and angiogenesis inhibition with drugs such as bevacizumab might be helpful in the future.

Shinohara et al23  reported durable remission after the administration of rituximab for EBV-negative, diffuse large B-cell lymphoma following autologous peripheral blood stem cell transplantation for angioimmunoblastic T-cell lymphoma.

Surgical Care

In a few cases, the removal of the spleen has improved the symptoms of AILD or induced remission. In addition, Nakashima et al24 reported successful coil embolization of a ruptured hepatic aneurysm in a patient with polyarteritis nodosa accompanied by angioimmunoblastic T cell lymphoma.

Consultations

This disease requires consultations from all specialists who can deal with specific manifestations of AILD. The primary doctor that should supervise care is an oncologist/hematologist. Because most patients die from infectious complications, an infectious disease specialist should also be involved.

Medication

The first-line agent in the treatment of AILD is prednisone. Interferon alfa is a promising agent in the treatment of AILD. Many agents have been used to treat AILD, but none is universally or consistently effective. The dosing regimens of these treatments have not been definitively set and hence are not included.

More on Angioimmunoblastic Lymphadenopathy With Dysproteinemia

Overview: Angioimmunoblastic Lymphadenopathy With Dysproteinemia
Differential Diagnoses & Workup: Angioimmunoblastic Lymphadenopathy With Dysproteinemia
Treatment & Medication: Angioimmunoblastic Lymphadenopathy With Dysproteinemia
Follow-up: Angioimmunoblastic Lymphadenopathy With Dysproteinemia
References
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References

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Further Reading

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Keywords

AILD, angioimmunoblastic T-cell lymphoma, diffuse plasmacytic sarcomatosis, immunoblastic lymphadenopathy, lymphogranulomatosis X, immunologic aberrations in idiopathic reticulosis, angioimmunoblastic lymphadenopathy, AIL

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Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Medical Editor

Evan R Farmer, MD, Professor of Dermatology, Johns Hopkins University School of Medicine, Clinical Professor of Pathology, Virginia Commonwealth University School of Medicine; Consulting Staff, Department of Dermatology, Johns Hopkins Hospital, VCU Health Services
Evan R Farmer, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, and International Society of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Daniel S Loo, MD, Associate Professor, Residency Program Director, Department of Dermatology, Boston University School of Medicine
Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology and Association of Professors of Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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