eMedicine Specialties > Dermatology > Lymphoma and Related Processes

Angioimmunoblastic Lymphadenopathy With Dysproteinemia: Follow-up

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

Follow-up

Further Inpatient Care

  • In patients with AILD, possible infections must be assessed and proper treatment for the infections that AILD sets the stage for with its accompanying immunosuppression must be treated.

Further Outpatient Care

  • Patients must be monitored for infections and informed that they must be evaluated for infections if they have a fever or other constitutional symptoms.

Inpatient & Outpatient Medications

  • Patients who are treated with chemotherapy must maintain their regimens and take proper medications for infections that can accompany AILD and chemotherapy.

Complications

  • Most patients die of infections due to immunologic compromise.

Prognosis

  • With treatment, remission occurs in about 25% of patients. However, most patients die from AILD. Overall, AILD has a moderately aggressive course, with occasional spontaneous remissions or protracted responses to therapy. The median survival is 24 months. AILD can evolve into high-grade lymphomas of T- or B-cell type, EBV-positive B-cell lymphomas, and chronic lymphatic leukemia (CLL) among other kinds of lymphoma and leukemia.
    • In 1995, in a univariate analysis, Siegert et al found that survival was significantly related to the following: age (P = .032), stage (P = .037), rash/pruritus (P = .038), edema (P = .030), ascites (P = .013), number of clinical symptoms (P = .004), LDH level (P = .007), and hemoglobin (P = .020).
    • In 1996, Schlegelberger et al25 found that certain cytogenetic findings were associated with a significantly lower incidence of therapy-induced remissions and a significantly shorter survival duration. Specifically, these cytogenetic findings included the presence of aberrant metaphases in unstimulated cultures; clones with an additional X chromosome; structural aberrations of the short arm of chromosome 1, preferentially involving 1p31-32; and complex aberrant clones with more than 4 aberrations.

Patient Education

  • Patients and their families must understand that AILD is usually a fatal disease and is accompanied by many potentially serious infections.
  • For excellent patient education resources, see eMedicine's Cancer and Tumors Center. Also, see eMedicine's patient education article Lymphoma.

Miscellaneous

Medicolegal Pitfalls

  • AILD must be distinguished from reactive processes that mimic it.
  • AILD is usually diagnosed at advanced stages because its symptoms are nonspecific; thus, in older people so-called B symptoms (ie, fever, weight loss, lymphadenopathy) should prompt investigation for AILD, leukemia, and lymphoma.

Special Concerns

  • Reactive processes can mimic AILD. Moreover, infections can occur with AILD. AILD can evolve into high-grade lymphoma. These situations should be considered when assessing and treating AILD.
 


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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