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Immune deficiency with polymorphic reticulosis.

T Najjar¹, C Gadol, M Y Khan

¹Oral & Maxillofacial Surgery & Pathology, University of Medicine and Dentistry, New Jersey Dental School, Newark 07103-2425.

Oral Surgery, Oral Medicine, and Oral Pathology

|March 1, 1989

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A fatal case of polymorphic reticulosis, a type of T cell lymphoma, presented initially as facial swelling and a lung nodule. Autopsy revealed disseminated cryptococcosis and Pneumocystis pneumonia due to severe immunodeficiency.

Area of Science:

Oncology
Infectious Diseases
Immunology

Background:

Polymorphic reticulosis is a rare condition now classified as a peripheral T cell lymphoma.
Peripheral T cell lymphomas (PTCLs) are aggressive cancers originating from mature T lymphocytes.
Immunodeficiency can predispose individuals to opportunistic infections and certain malignancies.

Observation:

A 40-year-old woman presented with nasolabial swelling and a pulmonary nodule.
The initial presentation mimicked less severe conditions, delaying definitive diagnosis.
The patient's condition rapidly progressed, leading to a fatal outcome.

Findings:

Autopsy confirmed polymorphic reticulosis (PTCL) as the underlying malignancy.
Disseminated cryptococcosis and Pneumocystis carinii pneumonia were identified.

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A secondary, severe immunodeficiency of unknown etiology was associated with the observed infections.

Implications:

This case highlights the varied presentations of peripheral T cell lymphoma.
It underscores the critical link between severe immunodeficiency and opportunistic infections.
The findings emphasize the importance of considering underlying immunosuppression in patients with aggressive lymphomas and infections.