|Year : 2016 | Volume
| Issue : 2 | Page : 229-232
Eosinophilic infiltration in lymph node in a child with idiopathic hypereosinophilic syndrome
Monika Garg, Ivreet Kaur, Kriti Chauhan, Charu Batra Atreja, Ridhima Auplish, Kusum Joshi
Department of Pathology, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, Haryana, India
|Date of Web Publication||20-Dec-2016|
Department of Pathology, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, Haryana
Source of Support: None, Conflict of Interest: None
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 Oct 20];4:229-32. Available from: http://www.amhsjournal.org/text.asp?2016/4/2/229/196209
| Introduction|| |
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. As there is no specific test for HES, it is a diagnosis of exclusion. It is a rare disorder in children. Most of the cases have been reported in women aged 20–50 years. Target organ damage mediated by eosinophilia is highly variable with the involvement of heart, skin, lung, central, and peripheral nervous system. 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|| |
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].
No history of dyspnea, orthopnea, wheezing, joint pains, oliguria, anuria, worm infestation, or allergic disorders.
The child was initially treated by prednisolone after which he became asymptomatic.
Multiple enlarged lymph nodes including right cervical and bilateral axillary region lymph nodes varying in size from 1.0 to 1.5 cm.
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.
Liver and pulmonary function tests were unremarkable.
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.
Negative for ß-hemolytic streptococci or Staphylococcus aureus.
Negative for any parasitic ova/cyst.
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).
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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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|| |
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. Prevalence of HES is unknown. 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. 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.,,
The HESs working group reached a consensus in 2005 and divided the heterogeneous group of chronic eosinophil-mediated disorders into six subgroups., FIPL1-PDGFR associated HES, chronic eosinophilic leukemia with increased blasts, lymphocytic HES with reactive eosinophilia, myeloproliferative-HES, organ-restricted eosinophilic disease and idiopathic HES. 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.
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. 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. Our patient responded well to steroids. The reason can be the absence of cardiologic or neurologic dysfunction at onset.
| Conclusion|| |
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]