Unicentric Castleman Disease Presenting as Persistent Cervical Lymphadenopathy: A Case Report

Abstract:

Cervical lymphadenopathy is a common clinical presentation with a wide differential diagnosis ranging from benign reactive conditions to malignant disorders. Among malignant causes, non-Hodgkin lymphoma represents a significant proportion, particularly in adults presenting with painless, persistent lymph node enlargement. We report the case of a 41-year-old woman who presented with progressive, painless right-sided cervical lymphadenopathy and severe anemia. Radiological evaluation revealed multiple enlarged cervical lymph nodes, and excisional biopsy confirmed the diagnosis of non-Hodgkin lymphoma. The patient was stabilized with supportive therapy and subsequently treated with systemic chemotherapy, resulting in significant clinical improvement and regression of lymphadenopathy. This case highlights the importance of early evaluation and histopathological confirmation of persistent lymphadenopathy in adults. Prompt diagnosis allows timely initiation of therapy, which is essential for improving outcomes in lymphoid malignancies. Reporting such cases from low- and middle-income countries is important to increase clinical awareness and contribute to regional data on disease presentation and management.

Keywords: Castleman disease, Cervical Lymphadenopathy, Lymph node biopsy, Malignancy

Introduction

Lymphadenopathy is a common clinical finding encountered in both primary and tertiary healthcare settings and may arise from a wide range of benign and malignant conditions. While most cases are reactive and self-limiting, particularly in younger individuals, persistent, painless lymphadenopathy in adults should raise suspicion for an underlying malignancy, especially lymphoma. ¹ Early recognition of this clinical sign is essential, as delayed diagnosis can adversely affect prognosis.

Lymphomas are malignant neoplasms of the lymphoid system and are broadly classified into Hodgkin lymphoma and non-Hodgkin lymphoma (NHL) based on histopathological, immunophenotypic, and clinical features. ² Non-Hodgkin lymphoma represents a heterogeneous group of disorders originating from B cells, T cells, or natural killer cells and exhibits considerable variation in biological behavior, ranging from indolent to highly aggressive forms. ³ Unlike Hodgkin lymphoma, NHL frequently presents extranodal involvement and disseminated disease at diagnosis.

Globally, lymphoma ranks among the ten most common malignancies, with non-Hodgkin lymphoma accounting for approximately 85–90% of all lymphoma cases. ⁴ Epidemiological data indicate a gradual increase in the incidence of NHL over recent decades, attributed to factors such as population aging, improved diagnostic techniques, chronic immune stimulation, environmental exposures, and the rising prevalence of immunodeficiency states. ⁵ Clinically, patients commonly present with painless lymphadenopathy, often involving the cervical region, along with systemic “B symptoms” such as fever, night sweats, unexplained weight loss, and fatigue. Hematological abnormalities, including anemia and other cytopenias, may occur due to bone marrow infiltration or chronic disease processes. ⁶

In Bangladesh, lymphoma is not considered a rare malignancy; however, accurate population-based prevalence data remain limited due to the lack of comprehensive cancer registries. ⁷ Available hospital-based studies suggest that non-Hodgkin lymphoma constitutes a substantial proportion of hematological malignancies diagnosed in tertiary care centers. ⁸ Patients frequently present at an advanced stage, largely due to delayed healthcare-seeking behavior, limited access to specialized diagnostic facilities, and low public awareness. Diagnosis relies primarily on imaging studies and histopathological examination of excised lymph nodes, which remains the gold standard. Treatment typically consists of combination chemotherapy, with or without immunotherapy, depending on disease subtype and stage. ⁹ This case report aims to highlight the clinical presentation, diagnostic approach, and management of non-Hodgkin lymphoma in a middle-aged woman from Bangladesh, emphasizing the importance of early recognition and timely intervention in resource-limited settings.

Case Presentation

A 41-year-old woman presented with a gradually progressive, painless swelling on the right side of the neck for several weeks. There was no history of trauma, and constitutional symptoms such as fever, night sweats, or significant weight loss were not clearly reported at initial presentation. Physical examination revealed multiple enlarged cervical lymph nodes, predominantly on the right side, which were firm, non-tender, and partially mobile. There was no documented hepatosplenomegaly. Initial laboratory investigations showed severe anemia with a hemoglobin level of approximately 5.4 g/dL, while total leukocyte and platelet counts were within normal limits. Inflammatory markers were elevated, and serum lactate dehydrogenase was raised. Renal function tests and serum electrolytes were within normal ranges. Computed tomography of the neck demonstrated multiple enlarged lymph nodes involving bilateral cervical regions, more prominent on the right, with loss of normal fatty hilum, raising suspicion of a lymphoproliferative disorder. Excision biopsy of a cervical lymph node revealed effacement of normal nodal architecture with sheets of atypical lymphoid cells, consistent with malignant lymphoma, and immunohistochemical findings supported a diagnosis of non-Hodgkin lymphoma. Baseline echocardiography showed normal cardiac chamber sizes and preserved left ventricular systolic function with an ejection fraction of approximately 68%, indicating suitability for systemic therapy.

The patient was initially stabilized with blood transfusions for severe anemia and subsequently started on standard chemotherapy under oncological care. On follow-up, there was significant regression of cervical lymphadenopathy and improvement in hemoglobin levels, with the patient tolerating treatment well and remaining clinically stable.

Table 1: Summary of Investigations

Investigation	Result	Interpretation
Hemoglobin	~5.4 g/dL	Severe anemia
Total WBC count	Within normal limits	Normal
Platelet count	Within normal limits	Normal
ESR	Elevated	Inflammation
Serum LDH	185 U/L	Elevated
Serum creatinine	0.83 mg/dL	Normal
Serum sodium	135 mmol/L	Normal
Serum potassium	3.8 mmol/L	Normal
CT scan (neck)	Multiple enlarged cervical lymph nodes (R > L)	Suspicious for lymphoma
Lymph node biopsy	Malignant lymphoma	Diagnostic
Echocardiography	EF ~68%	Normal systolic function

Outcome

Following confirmation of the diagnosis and initiation of systemic chemotherapy, the patient demonstrated a favorable clinical response. There was a marked reduction in the size of the cervical lymph nodes after the initial cycles of treatment, accompanied by improvement in constitutional symptoms and general wellbeing. Hemoglobin levels gradually improved with supportive care and treatment of the underlying disease, and no major hematological or systemic complications were observed during the early treatment period. The patient tolerated chemotherapy well, with no significant adverse effects necessitating treatment interruption. On follow-up visits, she remained clinically stable, with sustained regression of lymphadenopathy and improvement in functional status. The patient continues to receive regular oncological follow-up for monitoring of treatment response and early detection of potential relapses or therapy-related complications.

Discussion:

The present case describes a middle-aged woman presenting with persistent, painless cervical lymphadenopathy associated with severe anemia, ultimately diagnosed as non-Hodgkin lymphoma. This clinical presentation is consistent with existing literature, which emphasizes that painless, progressively enlarging lymph nodes in adults are a classical and often early manifestation of lymphoma. ¹⁰ Cervical lymph node involvement is particularly common and frequently serves as the initial reason for medical consultation, as observed in this patient. ¹¹

Severe anemia at presentation, as seen in this case, is a recognized but often underappreciated feature of non-Hodgkin lymphoma. Previous studies have reported anemia in a significant proportion of patients at diagnosis, attributable to bone marrow infiltration, chronic inflammation, nutritional deficiency, or cytokine-mediated suppression of erythropoiesis. ¹² The presence of anemia has also been associated with advanced disease and poorer prognosis in several cohorts, highlighting the importance of early evaluation. ¹³ Elevated lactate dehydrogenase (LDH), noted in this patient, further supports a high tumor burden and has been widely reported as an adverse prognostic indicator in lymphoma patients. ¹⁴

Radiological findings in this case demonstrated multiple enlarged cervical lymph nodes with loss of normal fatty hilum, features that are strongly suggestive of malignant lymphadenopathy. Similar imaging characteristics have been documented in previous studies, where nodal enlargement with architectural distortion correlated with histopathological confirmation of lymphoma. ¹⁵ However, imaging alone is insufficient for definitive diagnosis, underscoring the importance of excisional lymph node biopsy, which remains the gold standard for diagnosing lymphoma, as was appropriately performed in this case. ¹⁶

Histopathological examination revealed effacement of nodal architecture with atypical lymphoid proliferation, consistent with non-Hodgkin lymphoma. Comparable histological findings have been reported in multiple studies from both high- and low-income countries, reinforcing the universal diagnostic criteria for NHL regardless of geographic setting. ¹⁷ Baseline cardiac assessment showing preserved ejection fraction allowed safe initiation of chemotherapy, in line with international recommendations for pretreatment evaluation. ¹⁸

The favorable clinical response observed following chemotherapy, including regression of lymphadenopathy and improvement in hemoglobin levels, aligns with outcomes reported in similar cases where early initiation of standard combination chemotherapy resulted in significant disease control. ¹⁹ In Bangladesh, delayed presentation remains a common challenge, often leading to advanced-stage disease at diagnosis. ²⁰ This case highlights the importance of clinical vigilance and timely referral, demonstrating that appropriate diagnosis and treatment can lead to good short-term outcomes even in resource-limited settings.

Conclusion:

Early recognition of persistent, painless lymphadenopathy in adults is essential, as it may represent an underlying lymphoid malignancy requiring prompt evaluation. Timely histopathological confirmation allows appropriate risk stratification and early initiation of therapy, which are critical determinants of outcome. Recent advances in chemotherapy, immunotherapy, and targeted treatment options have transformed the management of non-Hodgkin lymphoma, offering improved survival even in rare and aggressive subtypes. Ongoing research, wider availability of modern therapies, and strengthening of diagnostic and oncology services in resource-limited settings provide optimism for better treatment responses, long-term disease control, and enhanced quality of life for affected patients.

Author of this Article

Dr. Tasnim Mahmud, MBBS, MPH, PGD (India), Department of Public Health, North South University.

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