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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 4  |  Issue : 2  |  Page : 229-232

Eosinophilic infiltration in lymph node in a child with idiopathic hypereosinophilic syndrome


Department of Pathology, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, Haryana, India

Date of Web Publication20-Dec-2016

Correspondence Address:
Kriti Chauhan
Department of Pathology, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-4848.196209

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  Abstract 

Hypereosinophilia is a frequent symptom that may occur in course of many underlying diseases. Hypereosinophilic syndrome (HES) is characterized by following criteria-Eosinophil count greater than 1500 μl persisting longer than 6 months and single or multiple organ system dysfunction attributable to cytotoxic injury by eosinophils without an identifiable etiology to explain eosinophilia. We present a case of HES with peripheral eosinophilia showing eosinophilic infiltration in lymph node. In an eleven year old boy who presented to Respiratory medicine OPD with intermittent fever and papular skin rashes.

Keywords: Hypereosinophilic syndrome, lymphadenopathy, sinus histiocytosis


How to cite this article:
Garg M, Kaur I, Chauhan K, Atreja CB, Auplish R, Joshi K. Eosinophilic infiltration in lymph node in a child with idiopathic hypereosinophilic syndrome. Arch Med Health Sci 2016;4:229-32

How to cite this URL:
Garg M, Kaur I, Chauhan K, Atreja CB, Auplish R, Joshi K. Eosinophilic infiltration in lymph node in a child with idiopathic hypereosinophilic syndrome. Arch Med Health Sci [serial online] 2016 [cited 2017 May 24];4:229-32. Available from: http://www.amhsjournal.org/text.asp?2016/4/2/229/196209


  Introduction Top


Idiopathic hypereosinophilic syndrome (HES) represents a heterogeneous group of leuko proliferative disorders associated with prolonged eosinophilia of undetectable cause with multiorgan system dysfunction. In this, the eosinophil count should exceed 1.5 × 109/L, persisting for at least 6 months unless death intervenes and there should be no evidence of eosinophil clonality.[1] As there is no specific test for HES, it is a diagnosis of exclusion. It is a rare disorder in children.[2] Most of the cases have been reported in women aged 20–50 years.[3] Target organ damage mediated by eosinophilia is highly variable with the involvement of heart, skin, lung, central, and peripheral nervous system.[4] Isolated involvement of lymph node is not documented. We present a rare case of idiopathic HES presenting with lymphadenopathy in a young child.


  Case Report Top


Presenting complaints

A 7-year-old boy presented with complaints of intermittent fever, headache, body ache, itchy papular skin rashes with cervical and axillary lymphadenopathy since last 2 years [Figure 1].
Figure 1: Child with skin rashes on left elbow.

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Negative history

No history of dyspnea, orthopnea, wheezing, joint pains, oliguria, anuria, worm infestation, or allergic disorders.

Drug history

The child was initially treated by prednisolone after which he became asymptomatic.

Local examination

Multiple enlarged lymph nodes including right cervical and bilateral axillary region lymph nodes varying in size from 1.0 to 1.5 cm.

Hematologic investigations

Normal hemoglobin (13.6 g/dl), raised total leukocyte count (58,700/cumm). Absolute eosinophil count (AEC) was 49,308/cumm. Peripheral blood smear showed eosinophilia [Figure 2]. Bone marrow aspiration and biopsy were hypocellular (overall cellularity 30%–40%) with a mild increase in eosinophils and its precursors. Erythrocyte sedimentation rate and C-reactive protein levels were unremarkable.
Figure 2: Peripheral blood film revealing eosinophilia.

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Biochemical investigations

Liver and pulmonary function tests were unremarkable.

Serological investigations

Serum immunoglobulin E (IgE) levels were raised 1405 IU/ml (normal range 0–280 IU/ml). Antibody detection (enzyme-linked immunosorbent assay) for parasites including toxoplasmosis, hydatidosis, toxocariasis, and antigen detection for filariasis were negative. Serology for HIV, hepatitis B virus, hepatitis C virus, Epstein–Barr virus, and antineutrophil cytoplasmic antibody was negative.

Blood culture

Negative for ß-hemolytic streptococci or Staphylococcus aureus.

Stool examination

Negative for any parasitic ova/cyst.

Radiology

Chest X-ray and echocardiography were unremarkable.

On the basis of these investigations, any underlying causes of hypereosinophilia were ruled out (including allergic and parasitic disorders, solid and hematological malignancies, Churg-Strauss disease).

Cytology

Fine needle aspiration cytology from right cervical lymph node showed a polymorphic population of reactive lymphoid cells with interspersed atypical lymphoid cells in a background of lymphoglandular bodies [Figure 3].
Figure 3: Smear showing a polymorphic population of lymphocytes with scattered occasional atypical lymphoid cells (L and G, ×400).

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Histopathology

Partially maintained architecture of lymph node with reactive follicles and sinus histiocytosis. A portion of lymph node showed heavy infiltration by sheets of eosinophils with scattered histiocytes. No granulomas were seen [Figure 4].
Figure 4: High power view showing partially maintained architecture of lymph node revealing heavy infiltration by eosinophils (H and E, ×400).

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On the basis of histology, various differential diagnoses were considered such as Kimura's disease, dermatopathic lymphadenitis, langerhan's cell histiocytosis (LCH), Hodgkin's lymphoma (HL), and angioimmunoblastic T-cell lymphoma (ATL). The absence of any prominent vascular component ruled out Kimura's disease and ATL. Dermatopathic lymphadenitis was excluded on the basis of the absence of any pigment laden histiocytes in the paracortex. Immunohistochemistry (IHC) was performed to rule out LCH and HL. CD1a and CD30 were negative while CD15 was positive in granulocytes. No RS cells were seen [Figure 5],[Figure 6],[Figure 7].
Figure 5: High power view showing CD15 positive granulocytes (immunohistochemistry, ×400).

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Figure 6: High power view showing a negative CD30 stain (immunohistochemistry, ×400).

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Figure 7: High power view showing a negative CD1a immunostain (immunohistochemistry, ×400).

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On the basis of morphology and IHC, a final diagnosis of eosinophilic infiltration in lymph node in a case of HES was made.

Treatment and follow-up

The child was put on prednisolone (1 mg/kg/day) and followed up after 6 months with complete hemogram including PBF examination. AEC was found to be markedly decreased.

Ethics of publication

The procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation. The ethics committee approves of the study, and informed consent was taken from the child.


  Discussion Top


HES constitutes a rare and heterogeneous group of disorder defined as persistent and marked blood eosinophilia (>1.5 × 109/L for more than 6 consecutive months) associated with evidence of eosinophil-induced organ damage, where other causes of hypereosinophilia such as allergic, parasitic, and malignant disorders have been excluded from this study.[1] Prevalence of HES is unknown.[4] In 2001, the World Health Organization formulated the criteria that distinguished idiopathic HES from chronic eosinophilic leukemia and T-cell mediated hypereosinophilia. The main criteria were the absence of increased percentage of blasts and lack of clonality of eosinophils or lymphocytes.[5] Recent advances have established that hypereosinophilia may be due to the occurrence of an interstitial chromosomal deletion on 4q12 leading to the creation of the FIPL1-PDGFRA fusion gene or due to increased interleukin-5 production by clonally expanded T-cell population (lymphocytic variant), most frequently characterized by a CD3/CD4+ phenotype.[4],[6],[7]

The HESs working group reached a consensus in 2005 and divided the heterogeneous group of chronic eosinophil-mediated disorders into six subgroups.[8],[9] FIPL1-PDGFR associated HES, chronic eosinophilic leukemia with increased blasts, lymphocytic HES with reactive eosinophilia, myeloproliferative-HES, organ-restricted eosinophilic disease and idiopathic HES.[5] Myeloproliferative-HES is characterized by clonal expansion of myeloid lineage, dysplastic eosinophils in peripheral blood, and normal IgE levels. Most patients have anemia, thrombocytopenia, increased serum B12 levels and splenomegaly. In lymphocytic HES, multiple organ systems are usually affected, but cardiac involvement in the form of endomyocardial fibrosis, valvular diseases, and congestive cardiac failure results in significant morbidity in children.[10]

Diagnosis of HES relies on observation of persistent and marked eosinophilia responsible for target-organ damage and exclusion of underlying causes of hypereosinophilia (including allergic and parasitic disorders, solid and hematological malignancies, Churg-Strauss disease, and human T-lymphotropic virus infection). The initial step in our case was to exclude all known causes of HES. Various immunological tests were done to rule out any parasitic infestation. Various hematological malignancies described above were considered. PDGFR rearrangement is required to know eosinophil clonality. Finding more than 2% of blasts in peripheral blood would correspond with clonal hypereosinophilia. In bone marrow, signs of dysplasia in all lineages with an increase in a number of blast between 5% and 19% suggest clonal origin.[11] Our case did not show increased blasts and no signs of dysplasia were seen in bone marrow. On the basis of histopathology, several differentials were considered as discussed earlier. Patient of HES is treated with prednisolone with an initial dose of 1 mg/kg. Although 70% of patients respond, few patients experience relapse. Hence, second line drug is given in the majority of cases including interferon-alpha or hydroxyurea.[12] Our patient responded well to steroids. The reason can be the absence of cardiologic or neurologic dysfunction at onset.


  Conclusion Top


Complications of hypereosinophilia occur most commonly in the skin, lung, and gastrointestinal system. However, isolated involvement of lymph nodes is not documented. Hence, our case is a rare presentation.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: Analysis of fourteen cases with review of the literature. Medicine (Baltimore) 1975;54:1-27.  Back to cited text no. 1
    
2.
Venkatesh C, Mahender E, Janani S, Malathi S, Vijayakumar M, Nammalwar BR. Hypereosinophilic syndrome. Indian J Pediatr 2006;73:237-9.  Back to cited text no. 2
    
3.
Bhatnagar S, Patwari AK, Narayan S, Lalit S, Anand VK. Idiopathic hypereosinophilic syndrome. Indian Pediatr 1999;36:824-7.  Back to cited text no. 3
    
4.
Roufosse FE, Goldman M, Cogan E. Hypereosinophilic syndromes. Orphanet J Rare Dis 2007;2:37.  Back to cited text no. 4
    
5.
Bain BJ, Gilliland DG, Horny HP, Vardiman JW. Chronic eosinophilic leukaemia, not otherwise specified. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al., editors. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: International Agency for Research on Cancer; 2008. p. 51-3, 68-73.  Back to cited text no. 5
    
6.
Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 2003;348:1201-14.  Back to cited text no. 6
    
7.
Salomon J, Białynicki-Birula R, Woźniak Z, Baran E. Pachydermatous eosinophilic dermatitis. Acta Dermatovenerol Croat 2011;19:31-5.  Back to cited text no. 7
    
8.
Helbig G, Wieczorkiewicz A, Dziaczkowska-Suszek J, Majewski M, Kyrcz-Krzemien S. T-cell abnormalities are present at high frequencies in patients with hypereosinophilic syndrome. Haematologica 2009;94:1236-41.  Back to cited text no. 8
    
9.
Klion AD, Bochner BS, Gleich GJ, Nutman TB, Rothenberg ME, Simon HU, et al. Approaches to the treatment of hypereosinophilic syndromes: A workshop summary report. J Allergy Clin Immunol 2006;117:1292-302.  Back to cited text no. 9
    
10.
Alfaham MA, Ferguson SD, Sihra B, Davies J. The idiopathic hypereosinophilic syndrome. Arch Dis Child 1987;62:601-13.  Back to cited text no. 10
    
11.
Peros-Golubicić T, Smojver-Jezek S. Hypereosinophilic syndrome: Diagnosis and treatment. Curr Opin Pulm Med 2007;13:422-7.  Back to cited text no. 11
    
12.
Hui CK. A case of dermatopathic lymphadenopathy associated with hypereosinophilic syndrome. Ann Acad Med Singapore 2012;41:35-6.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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