hodgkin lymphoma in children with case presentation

childhood Hodgkin lymphoma includes swollen lymph nodes, fever, drenching night sweats, and weight loss. Tests that examine the lymph system and other parts of the body are used to diagnose and stage childhood Hodgkin lymphoma. Certain factors affect prognosis (chance of recovery) and treatment options.Hodgkin lymphoma is a rather rare malignancy in the pediatric population, however, it constitutes approximately 40% of all lymphomas that present during childhood and is the most common malignancy in adolescents and young adults. In all age groups, Hodgkin lymphoma is highly sensitive to chemotherapy and irradiation

1. Submitted by Joel Rajan U Hodgkin lymphoma in children with case presentation

2. Hodgkin lymphoma was considered a uniformly fatal cancer { Hodgkin lymphoma is a type of cancer in the lymphatic system } Hodgkin described the disease in 1832, but most attributed its pathophysiology to an inflammatory condition. Formally known as Hodgkin disease, Hodgkin lymphoma (HL) is a highly curable malignancy. A unique neoplasm in which the malignant cell, the Reed-Sternberg cell (RSC), represents only a small proportion of cells constituting the bulk of the tumour. INTRODUCTION

3. Hodgkin’s disease (HD) is a malignant disorder of lymphoreticular system; a lymphoreticular neoplasm primarily of B cell lineage involving lymph nodes and the lymphatic system has unique molecular, histologic, immune phenotypic and clinical features.

4. The combination of I mmune deficiencies (from inherited conditions, medical treatment, or HIV infection)  some   Epstein-Barr virus (EBV) also will cause . Higher incidence in twins may be some of the other contributing factors Variation in the incidence of HD in different ethnic groups and association with human leukocyte antigen suggests that inherited susceptibility plays an important role in the pathogenesis ETIOLOGY

5. Nodular lymphocytic-predominant Hodgkin lymphoma (NLPHL) Patients with NLPHL generally present with localized, non-bulky disease. The only symptom for most people with NLPHL is one or more lumps. These are enlarged lymph nodes (swollen glands). They are often in only one place in the body. A few people have other general symptoms of lymphoma, like night sweats, weight loss and fevers Classical Hodgkin lymphoma The hallmark of classic Hodgkin lymphoma is the Reed Sternberg cell. This is a binucleated or multinucleated giant cell that is often characterized by a bilobed nucleus, with two large nucleoli, giving an owl’s eye appearance to the cells. Who Classification

6. Painless , movable lymph nodes in tissues surrounding involved area Unexplained fever Weight loss Drenching night sweats Malaise Painless cervical or supraclavicular lymphadenopathy Lymphadenopathy [ usually in the cervical, supraclavicular, and mediastinal areas; mediastinal presentation common in adolescents and young adults ] significant mediastinal adenopathy may cause cough , dyspnoea , or superior vena cava syndrome CLINICAL FEATURES

7. HIV Epstein-Barr virus infection C hronic infectious aetiologies such as Tuberculosis A bdominal lymphadenopathy Lymph proliferative conditions such as Castleman disease, Rosai -Dorfman Differential Diagnosis

8. Diagnosis Chest radiographic study to determine mediastinal or hilar node involvement Computed tomography to evaluate mediastinal, pulmonary, and abdominal disease Gallium and/or positron emission tomography (PET) scan to determine the extent of involvement Excisional lymph node biopsy essential to diagnosis and staging Bone marrow biopsy if patient has stage 3 or 4 disease according to imaging studies

9. TREATMENT Treatment modalities have varied from total nodal radiation therapy to chemotherapy to combination of chemotherapy and radiotherapy with significant improvement in survival rate throughout the last three decades. All children generally receive combination chemotherapy as initial treatment. Radiation therapy can cause profound musculoskeletal growth retardation and increase the risk for cardiovascular disease and secondary solid malignancies in children The volume of radiation and the intensity/duration of chemotherapy are determined by prognostic factors at presentation, including presence of constitutional symptoms, disease stage, and bulk.

10. Case Presentation

11. Case Report A 2-year 10-month-old child had presented with gradual onset of systemic symptoms over a period of 6–8 months The child was born at term. He was the first born of non-consanguineous parents. He had received all routine immunisations. However, he was not given the varicella vaccine . His developmental milestones were normal before he became ill. His growth parameters were between the 10th and 50th centile for his age until the age of 2 years. Subsequently, he had poor growth and it fell below the third centile when he was referred to us  was diagnosed as varicella infection, when he was about 2 years old

12. Symptoms W eight loss, Pallor, Intermittent fever an Recurrent abdominal pain. [multiple episodes of febrile illness along with abdominal pain during this period] The child had an episode of fever Skin r ash The child was having lack of appetite and poor weight gain D iarrhoea and Intermittent vomiting

13. Investigations H igh lactate dehydrogenase level 1632 U/L {normal less than 550} Absolute lymphopenia 930 cell/ μL T hrombocytosis (660×10 9 /L) H igh E rythrocyte S edimentation rate (70 in first hour.) a high C reactive protein level 78.2 mg/L { normal less than 5 mg/L} Liver function tests serum electrolytes, amylase, lipase and the coagulation profile were all normal T ests for infectious agents such as malaria, typhoid and dengue came out negative. Metabolic screening and blood culture reports were negative as well. Ultrasound-guided abdomen revealed abdominal lymph nodes contrast-enhanced CT of the abdomen could be localised in the retroperitoneal [ figure 2 ] and porta-hepatis regions as non-enhancing soft tissue masses [ figure 1 ] Laparoscopic biopsy of the nodes was undertaken, which on histological sections showed mixed cellularity-type Hodgkin's lymphoma

14.   The specimens (lymph node and the appendix) showed CD15, CD30 positivity in neoplastic Reed-Sternberg cells and leucocyte common antigen (CD45) and CD20 positivity in background cells, consistent with mixed cellularity diagnosis. Fluorodeoxyglucose-positron emission tomography (FDG-PET) scan ( figure 3 ) showed focal uptake of the tracer in supraclavicular, subcarinal, retroperitoneal area and liver, making this a stage IV disease, at presentation.

15. Figure 2 Contrast-enhanced CT of the abdomen showing retroperitoneal soft tissue mass Figure 1 Contrast-enhanced CT of the abdomen showing retroperitoneal mass compressing porta hepatis Figure 3 Positron emission tomography (PET) scan showing diffuse stage IV Hodgkin's lymphoma.

16. Final Diagnosis of Patient M ixed C ellularity-type Hodgkin's lymphoma Appendicitis

17. Treatment C hild received symptomatic treatment since he started to have clinical symptoms. These included multiple doses of antibiotics for his fever and intermittent infections. He was treated adequately with antigiardial therapy on the basis of the suspicion of giardiasis contributing to the partial duodenal villous atrophy detected by small intestinal biopsy. T he child was given packed cell transfusion, for severe anaemia Empirical antibiotics were started awaiting blood culture results and supportive measures were taken to control fever, maintain hydration and nutrition T he appendix was removed as it was found inflamed and enlarged. [ Subsequently, antibiotics were stopped and supportive treatment was continued]

18. Conclusion Hodgkin's lymphoma is rarely encountered in very young children. Primary involvement of the appendix in such cases has yet to be reported. This presents a diagnostic challenge. Differentiation from other chronic inflammatory and malignant conditions is essential to arrive at an accurate diagnosis. High index of clinical suspicion is required for early diagnosis and appropriate therapy.

19. 1.Natianal cancer institute https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq#:~:text=Signs%20of%20childhood%20Hodgkin%20lymphoma,of%20recovery)%20and%20treatment%20options 2. lymphoma action { https://lymphoma-action.org.uk/types-lymphoma-lymphoma-children-and-young-people/lymphoma-children } 3.Childrens cancer centre UK { https://www.childrenwithcancer.org.uk/childhood-cancer-info/cancer-types/hodgkin-lymphoma/ 4.NCBI { https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4602501/ } 5.Cancer Treatment centre {NCI} [ https://www.cancer.gov/types/lymphoma/patient/child-hodgkin-treatment-pdq#:~:text=Treatment%20of%20primary%20refractory%20or,the%20patient's%20own%20stem%20cells. 6.Lanzkowsky's Manual of Paediatric Haematology and Oncology 6th Edition [ https://t.me/Bjmedtech ] 7 . Palliative Care in Paediatric Oncology 1 st ed. 2018 Edition, Kindle Edition [ https://t.me/Bjmedtech ] 8. Paediatric Haematology and Oncology: Scientific Principles and Clinical Practice [ https://t.me/Bjmedtech ] 9. Oxford Textbook of Cancer in Children [ https://t.me/Bjmedtech ] 10. Manual of Paediatric Haematology and Oncology 4th Edition [ https://t.me/Bjmedtech ] Reference

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