|Year : 2021 | Volume
| Issue : 3 | Page : 346-349
Kimura's disease: A challenge to the cytologist
Department of Pathology, North Bengal Medical College, Darjeeling, West Bengal, India
|Date of Submission||24-Feb-2020|
|Date of Decision||21-Mar-2020|
|Date of Acceptance||24-Jun-2020|
|Date of Web Publication||10-Feb-2021|
Department of Pathology, North Bengal Medical College, Sushrutanagar, Darjeeling - 734 012, West Bengal
Source of Support: None, Conflict of Interest: None
Kimura's disease is a rare idiopathic chronic inflammatory disease which usually presents as deep-seated subcutaneous swelling in the head and neck region. Accompanied by lymphadenopathy, peripheral eosinophilia, and elevated immunoglobulin E levels, it is more prevalent among young Asian males. Here, I report the case of a 20-year-old male presenting with left posterior cervical lymph node swelling. Based on proper clinical setting and characteristic cytological features, a cytological diagnosis of Kimura's disease was rendered which was later confirmed by histopathological examination.
Keywords: Fine-needle aspiration cytology, immunoglobulin E, Kimura's disease
|How to cite this article:|
Chakrabarti I. Kimura's disease: A challenge to the cytologist. Med J DY Patil Vidyapeeth 2021;14:346-9
| Introduction|| |
Kimura's disease (KD) is a chronic, nonneoplastic, inflammatory disease of unknown cause, most often presenting as painless lymphadenopathy and/or tumor-like subcutaneous swelling predominantly in the head-and-neck region. They are often accompanied by blood eosinophilia and elevated serum immunoglobulin E (IgE) levels. Other than the head-and-neck region, cases have been reported with the involvement of the salivary gland, particularly the parotid, oral cavity, axilla, groin, and limbs., Coexisting renal disease is common, with an incidence ranging from 10% to 60%.
KD usually affects young males with the age range of 27–40 years, the male: female ratio being 3:1. More common in the Asian race, it is quite a rare disorder in the western part of the world. The first case of KD was reported by Kimm and Szeto from China in 1937, when they described seven such cases and named the condition as “eosinophilic hyperplastic lymphogranuloma.” It was in 1948, when Kimura et al. published its definitive histopathological description as an “unusual granulation combined with hyperplastic changes in lymphoid tissue.” Since then, the disorder came to be known by its present name as a tribute to Kimura et al.
KD is rare even in India, and its diagnosis on the basis of fine-needle aspiration cytology (FNAC) has rarely been described. Definitive diagnosis requires excisional biopsy in most of the cases. Early diagnosis is important to avoid unnecessary invasive diagnostic procedures.
| Case Report|| |
A 20-year-old male presented to the surgery outpatient department with an enlarged posterior cervical lymph node swelling on the left side of the neck for the past 6 months. The swelling measured 3 cm × 2.5 cm and was mobile, firm, and nontender on palpation. There was no other peripheral palpable lymph node or any organomegaly. The patient had no history of fever, loss of appetite, or weight loss. There was no history of cough, hemoptysis, or any contact with tuberculosis. The patient had a routine complete blood count report which showed 35% eosinophils, whereas the other parameters were within the normal range. He was investigated previously for supposed parasitic and allergic disorders, but all the results were nondiagnostic. The patient was sent to the Department of Pathology for IgE levels and FNAC. Following aspiration, the alcohol-fixed and air-dried smears were prepared and stained with Papanicolaou stain and Leishman–Giemsa stain, respectively. The smears showed a polymorphous population of reactive lymphoid cells of all generations along with histiocytes, plasma cells, and plenty of eosinophils. Some places even showed aggregates of eosinophils. There were also some fragments of fibrotic tissue and occasional clusters of the endothelial cells [Figure 1]. A single Warthin–Finkeldey giant cell was seen. Even after meticulous search, no Reed–Sternberg (RS) cell or any other atypical cell could be found. The IgE level was done in an outside laboratory, and the value was 1260 IU/ml. A provisional cytodiagnosis of KD was made, and excisional biopsy was advised for confirmation. The patient underwent lymph node excision after 2 months, and the tissue was sent for histopathological examination. The lymph node measured 3 cm × 3 cm and was solid and grayish on cut section. The section showed variable sized lymphoid follicles with enlarged germinal centers. Plenty of eosinophils and postcapillary proliferations were noted in the paracortical zone [Figure 2]. There were presence of eosinophilic abscesses as well, but Warthin–Finkeldey cells were not appreciated on the tissue section. The overall histopathological features were consistent with the cytodiagnosis of KD. The patient was, however, unfortunately lost on follow-up.
|Figure 1: Photomicrograph of cytological smears showing abundant eosinophils in a background of reactive lymphoid population, histiocytes, and plasma cells. (a) Papanicolaou stain, ×100 magnification, (b) Leishman–Giemsa stain, ×100 magnification|
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|Figure 2: Photomicrograph of histopathological sections showing. (a) Variably sized enlarged germinal centers (long arrows) (H and E, ×100) and (b) prominent eosinophilic infiltration and postcapillary proliferations (short arrows) in the paracortical zone (H and E, ×400)|
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| Discussion|| |
KD is a chronic inflammatory disease of unknown etiology. The probable causes include trauma, autoimmune reactions, allergic reactions to arthropod bites, parasites, viruses (Epstein-Barr virus and human herpes virus 8), fungus (Candida albicans), or some unknown toxins,, suggesting an abnormal immune response to an unknown stimulus. Some studies have also shown that the abnormal stimulation of CD4+ T cells, particularly CD4 T-helper2 cells and resultant overproduction of their cytokines, has triggered the formation of lymphoid follicles and high IgE levels., Thus, a Type IV type hypersensitivity reaction seems to be a more likely cause.
The diagnosis of KD by cytology is difficult, but with good clinical history and careful observation, the same can be rendered with somewhat certainty, thus avoiding more invasive diagnostic procedures. The clinical, cytological, and histopathological differentials include angiolymphoid hyperplasia with eosinophilia (ALHE), Hodgkin disease, drug reactions, allergic reactions to arthropod bites, Langerhans cell histiocytosis, and T-cell lymphomas. Castleman disease, dermatopathic lymphadenopathy, and Kaposi's sarcoma may also mimic this entity. However, a careful examination of cytology smears and proper clinical history and workup to rule out drug reaction, fungal or parasitic infection, and the possibility of arthropod bites often prove helpful.
The characteristic clinical findings coupled with eosinophilia, raised IgE levels, and the cytological features of reactive lymphoid hyperplasia with prominent eosinophilia, presence of endothelial cell clusters, Warthin–Finkeldey polykaryocytes, and fibrotic fragments point to the diagnosis toward KD. The absence of granulomas in cytology smears rule out allergic granulomatosis. Cases of T-cell lymphomas show atypical lymphocytes which are absent in KD. Hodgkin disease can be ruled out by the absence of the different types of RS cells depending on the variant, whereas Langerhans cell histiocytosis shows polygonal histiocytes with round, oval or bean-shaped nuclei and conspicuous longitudinal nuclear grooves. ALHE, also known as epithelioid hemangioma, has a more prominent vascular component and is the closest differential of KD. The two entities were once thought to be the spectrum of the same disease; however, the difference between the two is highlighted in [Table 1]. In short, smears from ALHE mostly show spindle-shaped, polygonal cells with vesicular nuclei and deeply eosinophilic cytoplasm containing well-defined vacuoles and plenty of immunoblasts along with eosinophils, plasma cells, and lymphocytes.
Thus, in a proper clinical setting, with adequate clinical history and workup, a classical case of KD can be diagnosed by FNAC smears with considerable accuracy by careful examination and ruling out the close differentials. Cell block preparation with subsequent immunohistochemistry for IgE and CD3 may also boost the confidence of cytodiagnosis of these cases. IHC with other specific antibodies will serve to differentiate KD from other differentials as well.
There is no standard guideline for the treatment, and the options include follow-up, surgical excision, steroids, and radiotherapy. However, the present case was unfortunately lost on follow-up, and hence, the response could not be studied.
| Conclusion|| |
In a characteristic clinical setting with hypereosinophilia and elevated IgE levels, KD can be diagnosed with considerable confidence on cytology smears. Early diagnosis prevents the necessity of invasive procedures.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
The author acknowledges the contribution of Dr. Amitabha Basu, Consultant Pathologist, Jalpaiguri Sadar Hospital, Jalpaiguri, West Bengal, India.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 1]