Angioimmunoblastic T-Cell Lymphoma Clinical Presentation

Updated: Apr 24, 2020
  • Author: Amira M Elbendary, MBBCh, MSc; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Angioimmunoblastic T-cell lymphoma (AITL) usually starts in a nonspecific fashion. Clinical manifestations are typical of lymphoma and may include the following:

  • Maculopapular rashes (these can resemble a viral rash)
  • Seropositive polyarthritis
  • Fever
  • Pruritus
  • Lymphadenopathy
  • Night sweats
  • Weight loss
  • Edema

As the disease progresses, patients may experience bone pain, ascites, and hepatosplenomegaly. Initially, AITL has a waxing and waning course; however, as it progresses, symptoms persist.

Patients can present with unusual infections. For example, Mönkemüller and Bronze reported a case of immunoblastic lymphadenopathy that presented as an acute abdomen and mixed bacteremia with Eikenella corrodens and group C Streptococcus infection. [18] Some patients have had disseminated infections with herpesvirus type 6 and other viral infections.

Incongruous clinical symptoms raise the possibility of AITL. For example, one reported case of AITL manifested as sick sinus syndrome, and others have had the appearance of collagen vascular diseases. Some patients have a history of collagen-vascular diseases, such as rheumatoid arthritis and dermatomyositis.

AITL can mimic tuberculosis. Singh et al reported a case of AITL with pulmonary involvement that was initially mistaken for tuberculosis based on fine-needle aspiration cytology and was treated with antituberculous therapy for 3 months. [19] The case was subsequently diagnosed as AITL based on lymph node biopsy results.

Hosoki et al described AITL developing with lymphocytic pleural effusion. [20]

Rare cases of AITL associated with proliferative glomerulonephritis have been reported. De Samblanx et al described a 67-year-old man with a nephrotic syndrome secondary to a proliferative glomerulonephritis, which was coincident with AITL. [21]

Batinac et al reported a case of AITL in an elderly patient who presented with generalized pruritic maculopapular eruption and fever after taking doxycycline. [22] Renner et al described eosinophilic cellulitis (Wells syndrome) in association with angioimmunoblastic lymphadenopathy. [23]

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Physical Examination

All organ systems can be affected by AITL. Typical findings at presentation include the following:

  • Rash (50% of cases)
  • Pruritus (30%)
  • Edema (40%)
  • Pleural effusion (40%)
  • Arthritis (20%)
  • Ascites (25%)

 Skin manifestations are the presenting sign of AITL in 40-50% of patients. Cutaneous involvement by AITL exhibits a highly variable presentation, typically with mixed features of an erythematous eruption with or without papules. The most commonly described lesions are a nonspecific morbilliform eruption mimicking a viral exanthem or drug eruption. Approximately 50% of patients with AITL-associated rash report pruritus. Other skin manifestations reported include nodules, annular lesions, and erythroderma. Vasculitis has been reported in AITL, including leukocytoclastic vasculitis, cutaneous medium-vessel vasculitis, and paraneoplastic vasculitis. [24]

There also are reports of patients with AITL with chronic urticarial eruptions. [24]

Skin laxity has been reported as a presenting sign of AITL. [25] Linear IgA dermatosis associated with cutaneous involvement of AITL has been reported. The patient presented with lesions that resembled toxic epidermal necrolysis, and immunofluorescence study revealed heavy IgA linear deposition in the dermoepidermal junction. [26]

Ferran et al suggested that the "deck-chair sign" (ie, rash with sparing of skin creases) is specific for cutaneous involvement by AITL. [27] AITL with scleromyxedemalike lesions and serum monoclonal protein has been reported, [28] as have subcorneal pustules and deep dermal-hypodermal nodules [29] and toxic epidermal necrolysis. [30]

Other possible findings include systemic lymphadenopathy, lacrimal and salivary gland involvement, and hepatosplenomegaly. Renal findings include renal amyloidosis, proliferative glomerulonephritis, and acute interstitial nephritis. Miura et al reported acute renal failure resulting from immunoglobulin M–lambda glomerular thrombi and membranoproliferative glomerulonephritis–like lesions in a patient with AITL. [31]

Pulmonary findings are varied and include hypoxemia. Some patients have interstitial pneumonia or bronchopneumonia. Patients with bronchopneumonia can have opportunistic infections, such as Pneumocystis jiroveci pneumonia; a case of cytomegalovirus infection has also been reported. Jarrett et al reported a case of shortness of breath and peripheral edema. [32]

Neurologic, rheumatologic, and related findings include retrobulbar neuritis, neuropathy, polyneuropathy, arthritis, papilledema, myelofibrosis, and inflammatory myopathy.

Goenka et al described a case of angioimmunoblastic lymphadenopathy with multiple polyps of the gastrointestinal tract. The patient presented with fever, abdominal mass, ascites, diarrhea, generalized lymphadenopathy, anemia, and marked peripheral eosinophilia. [33]

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Complications

Angioimmunoblastic T-cell lymphoma (AITL) is frequently associated with immunological abnormalities, including autoimmune cytopenias, hypergammaglobulinemia, and the presence of various autoantibodies. A few reports on AITL have also described the development of myelofibrosis. [34]

Pure red cell aplasia due to AITL has also been reported. [35]

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