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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.
Insights
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.
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.
Abstract:
A 77-year-old man presented with right inguinal lymphadenopathy and swollen parotid and submandibular glands bilaterally. Histopathology revealed germinal center B-cell type diffuse large B-cell lymphoma (DLBCL) in the inguinal lymph node. Lymphocyte and plasma cell infiltration in the submandibular gland with elevated serum IgG4 levels (13 g/L) prompted a diagnosis of IgG4-related disease (IgG4-RD). Systemic chemotherapy for DLBCL led to shrinkage of the lymph nodes and disappearance of the submandibular gland swelling, as confirmed by fluorodeoxyglucose-positron emission tomography/computed tomography. Although concomitant IgG4-RD and lymphoma have been reported, their simultaneous diagnosis is rare; therefore, a biopsy of all involved organs is crucial in cases with unusual organ involvement.
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