Angioimmunoblastic Lymphadenopathy with Dysproteinemia following Doxycycline Administration

Tumori - Tập 89 Số 1 - Trang 91-95 - 2003
Tanja Batinac1, Gordana Zamolo2, Nives Jonjić2, Franjo Gruber1, Bojan Miletić3, Irena Seili‐Bekafigo4, Miran Čoklo2
1Department of Dermatovenerology, Clinical Hospital Center;
2Department of Pathology, Medical Faculty, University of Rijeka;
3Department of Hematology, Clinical Hospital Center;
4Department of Internal Medicine, Clinical Hospital Center, Rijeka, Croatia

Tóm tắt

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a primary lymphoproliferative T-cell disorder, currently classified as a peripheral T-cell non-Hodgkin's lymphoma. AILD is characterized by generalized lymphadenopathy, hepatosplenomegaly, immunological abnormalities, polyclonal hypergammaglobulinemia and anemia. We report a case of AILD in an 80-year-old male who presented with a generalized pruritic maculopapular eruption and fever following doxycycline administration. The maculopapular rash progressed to formation of confluent nodules, plaques and finally erythroderma with lymphadenopathy and hepatosplenomegaly. Blood analysis revealed an elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. Lymph node biopsy showed almost complete effacement of the nodal architecture with diffuse proliferation of small vessels forming an arborizing network, surrounded by atypical lymphocytes, usually CD3+ CD4+ and occasionally CD3 + CD8+. There were also larger cells (immunoblastic shape) that displayed CD20 positively, some scattered plasma cells, and eosinophils. Histology of a cutaneous lesion showed spongiosis and infiltration of the epidermis by atypical lymphocytes with large hyperchromatic nuclei, perivascular dermal lymphocytic infiltrate (CD3+) mixed with plasma cells and occasional large immunoblasts (CD20+). During hospitalization the patient developed hemolytic anemia (Coombs positive) and lung metastases. The prognosis of AILD is generally poor, with a median survival of less than 20 months. Our patient died two and a half months after the diagnosis was made due to sepsis caused by Staphylococcus aureus isolated in hemoculture.

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Tumori

Tập 89 Số 1

91-95

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