Castleman disease in the neck with hypothyroidism and metabolic syndrome: a case report

Ya-Ling Li,Ying Zuo*

1Department of Endocrinology and Metabolism,Affiliated Hospital of North Sichuan Medical College,Nanchong 637000,Sichuan,China.

Abstract Castleman disease (CD) is a rare nonclonal lymph proliferative disorder of unknown etiology.Here,we report a case of multicentric Castleman disease (MCD) with hypothyroidism and metabolic syndrome presenting as a pain and slowly expanding,isolated neck mass in a 47-year-old Asian woman.It contributes to the practical aspects of diagnosis and treatment of Castleman disease in the neck.

Key words: Castleman disease,hypothyroidism,metabolic syndrome

Introduction

Castleman disease (CD) is a rare malignant disorder characterized by lymphocytes proliferation.CD can be divided into unicentric (UCD) and multicentric (MCD)types,based on the distribution of lymph nodes and organ involvement.According to the pathological results,it is be categorized as three types: hyaline vascular type,plasma cell type and mixed type.We herein report a newly-diagnosed case of MCD located in the neck with hypothyroidism and metabolic syndrome [1,2].

A 47-year-old Asian female patient presented with painless,slowly-enlarging thyroid and right-sided lymph nodes in the neck and was diagnosed of hypothyroidism through auxiliary clinical examination three years ago.This patient was admitted to our hospital on May 10,2010 due to pain and growing of thyroid and right-sided lymph nodes,puffiness of face,dyspnea after undergoing treatment of levothyroxine sodium tablets.The patient had no significant medical history and family history.The physical examination showed that Body Mass Index was 38.5kg/m2,Waist-to-hip ratio was 0.97,second degree thyroid enlargement with tenderness and multiple swollen lymph nodes in different sizes were detected in bilateral neck,supraclavicular fossa,axillary fossa,inguinal grooves.

The biochemical revealed triglyceride 3.14mmol/L,high-density-lipoprotein cholesterol 0.76mmol/L,uric acid 434.7umol/L,glucose 7.31mmol/L,C reaction protein 27.86mg/L.Her thyroid function showed free triiodothyronine 3.01pg/mL,free thyroxine 0.84ng/dl,hypersensitivity thyroid stimulating hormone 5.3030uIU/mL,thyroglobulin antibody 101.10U/ml,thyroid peroxidase antibody>1300.00U/ml,thyroglobulin>1000.00ng/ml.The glycated hemoglobin was 7.80% and oral glucose tolerance test(OGTT) detected diabetes (Table 1).It was negative for the human immunodeficiency virus (HIV) antibody,human herpesvirus 8 (HHV-8) and other laboratory examinations.Computed tomography (CT) scan indicated no abnormalities of the lungs,enlargement of thyroid and partially pressure and narrowing of the trachea.Positron emission tomography/computer tomography (PET/CT) showed that multiple lymph nodes were swollen throughout the body.Echocardiography displayed enlargement of left heart,mild mitral regurgitation and decreased diastolic function of left ventricle.B-ultrasound thyroid revealed swollen thyroid and suggested the possibility of Hashimoto's thyroiditis.The biopsy of her swollen lymph node proved MCD (Figure 1).

The patient underwent levothyroxine sodium tablet(50ug daily),liraglutide injection (0.6mg daily) in endocrinology.Initiated from June 2020,this patient was administrated dexamethasone injection (5mg once),prednisone treatment (40mg once) and rituximab injection (800mg once) in hematology every 4 weeks.Due to her personal circumstances,it was suspended until August,2020 and her treatment was changed to add cyclophosphamide for injection (1.6g once) to the original chemotherapy regimen.According to dynamically followed up her biochemical indicators and the B-ultrasound of lymph nodes,the patient was well tolerated with the treatment and lost five kilos so far (Table 2).

The Etiology of CD is unclear,and its clinical manifestations are variable.Based on the Mayo clinic study,84% of the patients had a recognized endocrinopathy.Among them,79% of patients have hypogonadism,54% of patients have endocrinopathies in the 4 major endocrine axes (gonadal,thyroid,glucose,and adrenal) and48% of patients have Abnormalities in glucose metabolism [3,4].

CD frequently affects lymphoid tissues of the thorax(70%),neck (15%),abdomen-pelvis (12%),and axilla(3%) [5].In this report,we described an uncommon presentation of MCD in the neck with hypothyroidism and metabolic syndrome.Accordingly,endocrinologists have to be followed up regularly and alert the missed diagnosis or misdiagnosis of CD,when the patients manifested the swollen of the neck or lymph nodes.The correct identification of CD can contribute to diagnosis and treatment timely.

Table 1.The result of OGTT.

Figure 1.Hematoxylin-eosin staining showed that the normal structure remained and a large number of plasma cells infiltrated in the interfollicular area.Immunohistochemical CD38 showed plasma cell proliferation.

Table 2.The biochemical indicators and the B-ultrasound of lymph nodes changes before and after treatment.