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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 15  |  Issue : 3  |  Page : 488-491

A rare case of kikuchi-fujimoto disease presenting as a mediastinal tumor with bilateral chylothorax


1 Department of Respiratory Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences (Deemed to be University), Wardha, Maharashtra, India
2 Department of Pathology, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences (Deemed to be University), Wardha, Maharashtra, India

Date of Submission27-Apr-2020
Date of Decision05-May-2020
Date of Acceptance20-Jun-2020
Date of Web Publication1-Feb-2021

Correspondence Address:
Dr. Babaji Ghewade
Prajakt, 165, Dreamland City, Sawangi Meghe, Wardha - 442 001, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdmimsu.jdmimsu_161_20

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  Abstract 


Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare, benign, and self-limiting condition with unknown etiology primarily affecting young women of Asian descent and is characterized by cervical lymphadenopathy. Diagnosis requires histopathologic examination and exclusion of other factors by ancillary studies. Non-Hodgkin lymphoma and systemic lupus erythematosus should be ruled out before a diagnosis of KFD, given the overlapped clinical and histologic features as well as the different therapeutic approaches. However, pleural effusion has rarely been reported in KFD patients. Here, we report an interesting case of an 18-year-old male who presented to us with features of cervical lymphadenopathy and fever who was later diagnosed as a case of Kikuchi-Fujimoto disease with bilateral chylothorax.

Keywords: Chylothorax, Kikuchi-Fujimoto disease, lymphadenopathy


How to cite this article:
Dubey A, Wankhade R, Ghewade B, Bhake A. A rare case of kikuchi-fujimoto disease presenting as a mediastinal tumor with bilateral chylothorax. J Datta Meghe Inst Med Sci Univ 2020;15:488-91

How to cite this URL:
Dubey A, Wankhade R, Ghewade B, Bhake A. A rare case of kikuchi-fujimoto disease presenting as a mediastinal tumor with bilateral chylothorax. J Datta Meghe Inst Med Sci Univ [serial online] 2020 [cited 2021 Feb 25];15:488-91. Available from: http://www.journaldmims.com/text.asp?2020/15/3/488/308548




  Introduction Top


 Kikuchi-Fujimoto disease More Details (KFD), also known as histiocytic necrotizing lymphadenitis, is an enigmatic, benign, and self-limiting condition characterized by regional lymphadenopathy, usually associated with mild fever and night sweats. It was first reported in Japan, almost simultaneously by Kikuchi and by Fujimoto and colleagues in 1972 as lymphadenitis with a focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris.[1] Although unknown infectious agents and an autoimmune response have been considered as the prime causes, the KFD pathogenesis remains unclear.[2]

The diagnosis of KFD still remains challenging because of its shared features with other lymph node diseases and continue to pose significant risks of misdiagnosis and unnecessary treatment.[3]


  Case Report Top


An 18-year-old male presented to our outpatient unit with complaints of dry cough, breathlessness, low-grade fever, difficulty in deglutition, and loss weight for 1 month. There was no history of tuberculosis or antitubercular treatment. His physical examination revealed tachycardia (heart rate = 112/min), tachypnea (respiratory rate = 24/min), and bilateral multiple cervicals and axillary lymphadenopathy. The palpable lymph nodes were firm, tender, and conglomerated, not fixed to the underlying structure or the overlying skin. The respiratory system examination revealed parasternal dullness on percussion and decreased breath sounds on auscultation. His hemogram and biochemical parameters were within normal limits except for raised lactate dehydrogenase (LDH) level (540 U/L) and raised erythrocyte sedimentation rate (ESR) (35 mm/h).

A routine chest X-ray was performed which revealed mediastinal widening, obliteration of the right costophrenic and cardiophrenic angles, and thickened right horizontal fissure suggestive of a mediastinal mass lesion with right-sided pleural effusion.[Figure 1].
Figure 1: Chest X-ray posteroanterior view suggestive of the anterior mediastinal mass lesion with right-sided pleural effusion

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The patient underwent a ultrasonography -guided diagnostic thoracentesis which revealed the pleural effusion to be a chylothorax [Figure 2].
Figure 2: Milky appearance of the pleural fluid aspirated from the right pleural space

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The pleural fluid was exudative by Light's criteria and pleural fluid findings included-ADA = 13.40 IU/L, CBNAAT = negative for MTB, no growth of any pathological organism on culture, and triglyceride = 124 mg/dl.

A follow-up, chest X–ray, was done 2 days after the thoracentesis which revealed increase in the amount of fluid in the right pleural space and appearance of pleural effusion in the left pleural space, and therefore an intercostal drainage tube was inserted in the right pleural space and the fluid was drained [Figure 3].
Figure 3: Chest X-ray posteroanterior view suggestive of bilateral pleural effusion with ICD tube in situ in the right pleural space

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The patient was then subjected to contrast-enhanced computed tomography scan of the thorax which was suggestive of extensive homogenously enhancing lymphadenopathy in mediastinum, cervical, supraclavicular region, and bilateral axilla (possibility of LYMPHOMA) with bilateral pleural effusion and passive atelectasis of basal segment of lower lobes [Figure 4].
Figure 4: A contrast-enhanced computed tomography scan of the thorax revealing an anterior mediastinal mass with bilateral pleural effusion

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The patient then underwent excision biopsy of the right supraclavicular lymph node. The affected node showed focal, well circumscribed, paracortical necrotizing lesions with abundant karyorrhectic debris, and scattered fibrin deposits. The plasma cells and neutrophils were very scanty, instead plasmacytoid monocytes were numerous. These findings were suggestive of the diagnosis of “Kikuchi Fujimoto Disease” [Figure 5] and [Figure 6].
Figure 5: Kikuchi-Fujimoto disease: Showing necrotizing change centered in the subcapsular region(HP, H and E, ×10)

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Figure 6: Kikuchi-Fujimoto disease: showing monocytoid plasma cells, histiocytes, polymorphous lymphocytes adjacent to the area of nonspecific necrosis containing pyknotic and karyorrhectic debris. No emperipolesis and no RS cells (HP, H and E, ×40)

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The patient was started on nonsteroidal anti-inflammatory drugs and glucocorticoids, in response to which the patient regained normal body temperature, the enlarged lymph nodes regressed in size and the pleural effusion was resolved partially. The patient was symptomatically better after 5 days of starting the treatment and was discharged. Unfortunately, the patient was lost to follow-up.


  Discussion Top


KFD was first reported in Japan in 1972. It is generally considered to be a nonneoplastic lymph node enlargement disease, a type of reactive lymphoid hyperplasia.[4] It mainly affects the young women (M:F = 1:3) of Asian descent, however, it has been identified in all ethnicities, ages, and in men. The cause of KFD is still unknown but a viral or autoimmune cause has been suggested. The microorganisms more frequently reported to have a causative role in KFD includes  Yersinia More Details enterocolitica, Toxoplasma gondii, Epstein–Barr virus, Human Herpesvirus 6 and 8, Hepatitis B virus, Parvovirus B19, herpes simplex virus, and cytomegalovirus.[1] KFD has nonspecific clinical features primarily fever and lymphadenopathy and therefore can be easily confused with other diseases.[2] Other extranodal findings in KFD comprise skin lesions (maculopapular lesion, morbilliform rash, urticaria, and malar rash), hepatosplenomegaly, and neurological involvement (meningitis and acute cerebellar syndromes) but these findings are not very common. In developing countries, prolonged fever and cervical lymphadenopathy are characteristic of tuberculosis infection, which is a major differential diagnosis of KFD. By contrast, in developed countries, KFD is often misdiagnosed as lymphoma, sarcoidosis, infectious mononucleosis, cat-scratch disease, some collagen diseases (mainly systemic lupus erythematosus), drug eruption, and other disorders.[5]

Isolated mediastinal lymphadenopathy without cervical lymph node swelling is very rare in KFD.[5] Our patient presented with cervical lymphadenopathy and anterior mediastinal lymphadenopathy with bilateral chylothorax. Laboratory studies in patients of KFD are fairly non-specific. The most common finding is leukopenia, followed by atypical lymphocytes; however, the majority of patients have a normal complete blood cell count. ESR also tends to be elevated in most patients and there may also be abnormalities in liver enzymes and an elevated LDH.[6] The incidence of pleural effusion in KFD is low and rarely reported clinically, so we cannot reach any conclusion regarding the likelihood that KFD patients might have pleural effusion. In the present case, the patient had an exudate and that too a chylothorax. The mechanism of its occurrence remains unclear but probably due to thoracic duct obstruction by enlarged mediastinal lymph nodes.[7]

The definitive diagnosis of KFD is made on histopathological examination of the lymph node biopsy specimen. The characteristic histopathologic findings of KFD include irregular paracortical areas of coagulative necrosis with abundant karyorrhectic debris, which can distort the nodal architecture, and large numbers of different types of histiocytes at the margin of the necrotic areas. Thrombosed vessels usually are seen around the areas of necrosis. The karyorrhectic foci are formed by different cellular types, predominantly histiocytes and plasmacytoid monocytes, but also immunoblasts, some of which may be atypical and small and large lymphocytes. Reactive lymphoid follicles are present in 50% to 60% of cases, and follicular hyperplasia has been found in approximately 10% of cases.[1]

The treatment of KFD is usually supportive. Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to alleviate lymph node tenderness and fever. The use of corticosteroids, such as prednisone, has been recommended in severe extranodal or generalized Kikuchi's disease. Immunosuppressive medications are recommended as an adjunct to corticosteroids in severe, life-threatening disease. Excisional lymph node biopsy for the purpose of confirming the diagnosis is the only surgery indicated in Kikuchi's disease.[8]

To conclude, KFD patients are often admitted in a pulmonary medicine department, and it is easy to misdiagnose them as having tuberculous pleurisy, lymphadenitis, or a lymphoid malignancy. We report this patient to improve clinician's understanding of such rare diseases and help reduce the likelihood of misdiagnosis.[9],[10],[11],[12],[13],[14],[15]

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease a comprehensive review. Am J Clin Pathol 2004;122:141-52.  Back to cited text no. 1
    
2.
Xu S, Sun W, Liu J. Kikuchi-Fujimoto disease: A case report and the evaluation of diagnostic procedures. BMC Oral Health 2019;19:223.  Back to cited text no. 2
    
3.
Lahma J, Arkoubi Z, Hejjouji R, Nitassi S, El Ayoubi A, Bencheikh R, et al. About a rare disease misdiagnosed as malignant lymphoma or tuberculosis: Kikuchi-Fujimoto's disease. Pan Afr Med J 2018;31:77.  Back to cited text no. 3
    
4.
Perry AM, Choi SM. Kikuchi-Fujimoto disease: A review. Arch Pathol Lab Med 2018;142:1341-6.  Back to cited text no. 4
    
5.
Hino H, Nishimura T, Nitadori J, Miyakoshi S, Seki A, Arai T, et al. An uncommon presentation of Kikuchi-Fujimoto disease as mediastinal lymphadenopathy. J Thorac Dis 2016;8:E330-3.  Back to cited text no. 5
    
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Humphreys S, Oikonomou KG, Ward N, Aye M. Kikuchi disease: The great masquerador-A case report and review of the literature. Med Case Rep 2018;4:62-5.  Back to cited text no. 6
    
7.
Liu X, Huang S, Jiang G. Report of a rare case of histiocytic necrotizing lymphadenitis with bilateral pleural effusion diagnosed via cervical lymph node biopsy. Sao Paulo Med J 2018;136:368-71.  Back to cited text no. 7
    
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Tati S, PBRK, EY, KP, KVVR. Kikuchi-Fujimoto disease?: A rare case representation and review of literature. J Evol Med Dent Sci 2015;4:2214-7.  Back to cited text no. 8
    
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Jain J, Banait S, Tiewsoh I, Choudhari M. Kikuchi's Disease (Histiocytic Necrotizing Lymphadenitis): A Rare Presentation with Acute Kidney Injury, Peripheral Neuropathy, and Aseptic Meningitis with Cutaneous Involvement. Indian J Pathol Microbiol 2018;61:113-15. Available from: https://doi.org/10.4103/IJPM.IJPM_256_17. [Last accessed on 2020 Feb 05].  Back to cited text no. 9
    
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Vadala R, Talwar D, Talwar D. Recurrent Non-Traumatic Idiopathic Chylothorax: A Diagnostic Dilemma with Therapeutic Challenge. Respirology Case Reports 2020;8:637-40. Available from: https://doi.org/10.1002/rcr2.637. [Last accessed on 2020 Feb 05].  Back to cited text no. 10
    
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Dhamgaye TM. In Reply: Latent TB Treatment in Pregnant Women: A Patient Perspective. Int J Tuberc Lung Dis 2020;24:1226-7. Available from: https://doi.org/10.5588/ijtld.20.0390. [Last accessed on 2020 Feb 05].  Back to cited text no. 11
    
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Dhamgaye TM, Bhaskaran DS. An Unusual Pulmonary Metastatic Manifestation of Gestational Choriocarcinoma: A Diagnostic Dilemma. Lung India 2017;34:490-1. Available from: https://doi.org/10.4103/lungindia.lungindia_77_14. [Last accessed on 2020 Feb 05].  Back to cited text no. 12
    
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Ganasathi K, Jadhav U, Belsare A. To Study the Utility of Fibreoptic Bronchoscopy in Diagnosis of Various Lung Conditions: A Study Protocol. Int J Pharm Res 2019;11:2022-5. Available from: https://doi.org/10.31838/ijpr/2019.11.04.504. [Last accessed on 2020 Feb 05].  Back to cited text no. 13
    
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Gupta V, Bhake A. Clinical and Cytological Features in Diagnosis of Peripheral Tubercular Lymphadenitis – A Hospital-Based Study from Central India. Indian J Tuberc 2017;64:309-13. Available from: https://doi.org/10.1016/j.ijtb.2016.11.032. [Last accessed on 2020 Feb 05].  Back to cited text no. 14
    
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Gupta V, Bhake A. Reactive Lymphoid Hyperplasia or Tubercular Lymphadenitis: Can Real-Time PCR on Fine-Needle Aspirates Help Physicians in Concluding the Diagnosis?. Acta Cytol 2018;62:204-8. Available from: https://doi.org/10.1159/000488871. [Last accessed on 2020 Feb 05].  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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