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Immunodeficiency Diseases

Immunodeficiency Diseases

Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency...

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IgG4-related Disease Concomitant with Diffuse Large B-cell Lymphoma.

Hiroto Yanagisawa¹, Keita Mishima¹, Yaya Yamanouchi¹

¹Department of Hematology & Immunology, Kanazawa Medical University, Japan.

Internal Medicine (Tokyo, Japan)

|August 7, 2024

Summary

This study reports a rare case of simultaneous IgG4-related disease (IgG4-RD) and diffuse large B-cell lymphoma (DLBCL) in a patient presenting with lymphadenopathy and gland swelling. Biopsies are crucial for diagnosing concurrent conditions.

Keywords:

IgG4-related disease diffuse large B-cell lymphoma

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Area of Science:

Oncology
Immunology
Pathology

Background:

Diffuse large B-cell lymphoma (DLBCL) and IgG4-related disease (IgG4-RD) are distinct conditions that can affect lymph nodes and salivary glands.
Simultaneous occurrence of DLBCL and IgG4-RD is uncommon, posing diagnostic challenges.

Purpose of the Study:

To report a rare case of concurrent DLBCL and IgG4-RD in a 77-year-old male.
To highlight the importance of comprehensive histopathological evaluation in cases with unusual organ involvement.

Main Methods:

Clinical presentation of right inguinal lymphadenopathy and bilateral parotid/submandibular gland swelling.
Histopathological examination of inguinal lymph node revealing germinal center B-cell type DLBCL.
Submandibular gland biopsy showing lymphocyte and plasma cell infiltration with elevated serum IgG4 levels, leading to IgG4-RD diagnosis.
Assessment of treatment response using fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT).

Main Results:

Diagnosis of DLBCL and IgG4-RD confirmed through biopsies.
Systemic chemotherapy for DLBCL resulted in significant reduction of lymphadenopathy and submandibular gland swelling.
FDG-PET/CT confirmed treatment efficacy.

Conclusions:

Concurrent DLBCL and IgG4-RD, while rare, necessitates thorough investigation.
Biopsy of all affected organs is critical for accurate diagnosis and management of simultaneous conditions.
This case underscores the complexity of diagnosing overlapping lymphoproliferative and autoimmune disorders.