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LYMPHOMA Introduction: Hodgkin's lymphoma was first described by Thomas Hodgkin, a British physician, in 1832. They are the 3rd most common malignancy in children. Over the last several decades, chemotherapy has revolutionized the treatment of pediatric lymphomas and cure is now possible for most...

LYMPHOMA Introduction: Hodgkin's lymphoma was first described by Thomas Hodgkin, a British physician, in 1832. They are the 3rd most common malignancy in children. Over the last several decades, chemotherapy has revolutionized the treatment of pediatric lymphomas and cure is now possible for most children suffering from lymphoma. Hodgkin lymphoma is a success story in pediatric oncology, with one of the best cure rates amongst all oncological disorders. Definition Lymphoma is cancer that begins in infection-fighting cells of the immune system, called lymphocytes. These cells are in the lymph nodes, spleen, thymus, bone marrow, and other parts of the body. So lymphoma is also well known as cancer of the lymphatic system. Types of Lymphoma It can be subdivided into 2 major subgroups: 1. Hodgkin Lymphoma 2. Non- Hodgkin Lymphoma Hodgkin Lymphoma Definition Hodgkin lymphoma, a lymphoreticular neoplasm primarily of B cell lineage involving lymph nodes and the lymphatic system Epidemiology:  Lymphomas account for approximately 10% of all pediatric malignancies. Hodgkin lymphoma contributes to about 40% of all pediatric lymphomas, occurring with a frequency of 5-7 per million.  In the developing world, the first peak is early in the pre-pubertal age group. Another peak occurs in adulthood, beyond 40-50 years of age. Hodgkin Lymphoma is unusual in younger 1  children below 5 years. A disproportionate sex distribution with male predominance has been reported. The reason for this is unknown.  In children younger than 10 years, the incidence of Hodgkin lymphoma is twofold to threefold higher in males than in females. In children aged 10 to 14 years, the incidence is approximately 1.2-fold higher in males than in females. Etiology The etiology has not been clearly elucidated.  However, infections like EBV( Epstein Barr Virus) and HIV are associated with an increased risk of the disease. EBV viral DNA can be found in Reed-Sternberg cells suggesting that monoclonal proliferation of the neoplastic clone takes place after EBV infection  Immunocompromised children with underlying diseases like ataxia-telangiectasia are more likely to be affected.  A familial component has been detected, with siblings being at a higher risk of disease. A strong evidence for genetic susceptibility comes from a 100-fold increased risk in monozygotic twins compared with dizygotic twins.  Immune deficiency and autoimmune conditions (rheumatoid arthritis, SLE, sarcoidosis) are known to be associated with increased risk of Hodgkin lymphoma. 2 Pathophysiology 3 Classification of Hodgkin lymphoma Hodgkin lymphoma can be classified into two major subtypes: (a) Classical HL and (b) nodular lymphocyte predominant HL. WHO classification of Hodgkin Lymphoma 1. Classical Hodgkin Lymphoma  Nodular Sclerosis subtype  Mixed Cellularity subtype  Lymphocyte- rich subtype  Lymphocyte – depleted subtype 2. Nodular Lymphocyte predominant Hodgkin Lymphoma 4 Types of Hodgkins Lymphoma 1. Classic Hodgkin's lymphoma: It is the more comm type of lymphoma. It accounts for > 9 in 10 cases of Hodgkin lymphoma in developed countries. Child diagnosed with classical Hodgkin lymphoma has large, abnormal cells called Reed-Sternberg cells in the lymph nodes. Subtypes of classic Hodgkin lymphoma a. Nodular sclerosis Hodgkin lymphoma: It is more common in teens and young adults but it can occur in any age. It has a variant of RS cell called lacunar cell. There is thickened capsule with proliferation of collagenous bands that divide the node into nodules. It tends to start in lymph nodes in the neck or chest. b. Mixed cellularity Hodgkin lymphoma: This is the second most common type, found in about 4 out 10 cases. It is seen mostly in people with HIV infection. It is also found in children or the elderly. It can start in any lymph node but most often occurs in the upper half of the body. Plenty of RS cells & numerous eosinophils, plasma cells, lymphocyte & benign histiocytes. c. Lymphocyte-rich Hodgkin lymphoma: It is not common and usually occurs in the upper half of the body. It is rarely found in more than a few lymph nodes. d. Lymphocyte-depleted Hodgkin lymphoma: This is a rare form of Hodgkin disease. It is seen mainly in older people and those with HIV infection. It is more aggressive than other types. It is most often found in lymph nodes in the abdomen, spleen, liver and bone marrow. Staging of Hodgkin lymphoma: Modified Ann-Arbor Staging for Hodgkin Lymphoma Stage Definition I Involvement of a single lymph node region (I) or of a single extralymphatic organ/site (IE) II Involvement of 2 or more lymph nodes regions on same side of diaphragm (II) or localized involvement of an extra lymphatic organ/site on same side of diaphragm(IIE) III 5  III Involvement of lymph node regions on both sides of diaphragm (III), may be accompanied by involvement of spleen (IIIS) or by localized involvement of an extra- lymphatic organ or site (IIIE) IV Diffuse or disseminated involvement of one or more extra lymphatic organ tissues with or without associated lymph node involvement. Clinical Features  The involved areas are typically lymph nodes, with contiguous spread to other areas.  The commonest symptom is development of slowly progressive lymphadenopathy.  The lymph nodes involved in HL are typically non-tender with a rubbery feel. Cervical lymph nodes are most commonly involved (75%) followed by mediastinal lymph nodes consists of (50%)  In advanced diseases, liver, spleen, bone marrow and other visceral organs may become involved.  Less commonly, axillary or inguinal lymphadenopathy Presence of B symptoms is important to further stage of the disease. ' B symptoms indicate an advanced stage of disease. B' symptoms are present if the patient has any of the following features:  Fever>100.4°F for at least 3 consecutive days,  drenching night sweats  Significant weight loss (>10%) in the last 6 months. Investigations 1. Careful history taking ,physical examination with assessment of all lymph node bearing areas 2. Lymph node biopsy.  Either excision or a core needle biopsy is sufficient. Fine needle aspiration cytology (FNAC) not recommended. 6   Tumor cells stain positive for CD15 and CD30, while negative for CD45. CD20 is variably positive. However, nodular lymphocyte predominant HL (NLPHL) has a very different picture, with cells staining positive for CD45, CD20, and negative for CD15 and CD30. Staging investigations 3. Complete CBC with ESR, C reactive protein 4. Liver and renal functions tests, alkaline phosphatase 5. Chest X-ray (PA and lateral views): Chest radiograph helps in assessing presence of mediastinal enlargement. 6. CT scan of the chest provides information about pulmonary parenchyma, chest wall, pleura and pericardium 7. Bone marrow biopsy (all children except stages IA/IIA) 8. Bone scan (if bone pain or raised serum alkaline phosphatase) 9. Abdominal ultrasound. 10. CT scan (neck to abdomen)  It is typically performed to evaluate lymph nodal groups, as well as the potential extra- nodal sites, including liver, spleen, intestine, and lung parenchyma. 11. Evaluation of bone marrow by doing both aspirate and biopsy is recommended for advanced stage HL, typically for disease stage IIB and beyond. In coming years, it is possible that PET and MRI may become a substitute for bone marrow examination. Treatment  Hodgkin Lymphoma is an exquisitely chemosensitive and radiosensitive disease with high chances of cure after appropriate therapy. Chemotherapy alone may be curative for several patients, especially those who have low risk disease, and show a good response to initial chemotherapy cycles.  ABVD regimen used to be the benchmark of therapy; however, it is no longer considered a preferred modality for children as it has significant long-term toxicity. (cardiomyopathy, pulmonary fibrosis) The advantage of this approach is elimination of radiation associated adverse effects like myocardial dysfunction, musculoskeletal growth deficits and second malignancy. 7   Unfavorable disease or localized disease with B symptoms are treated with 4-6 cycles of ABVD with/without radiotherapy  Some of the current treatment regimens include OEPA-COPDAC (Vincristine, Etoposide , Prednisone, Adriamycin-Cyclophosphamide, Vincristine, Prednisone, Dacarbazine), or AV-PC (Adriamycin, Vinblastine, Prednisone, Cyclophosphamide).  In early stage, 3-4 cycles of chemotherapy alone may work well  Bulky disease may be another reason to add radiotherapy.  The role of additional radiotherapy in stage III and IV disease remains controversial. Adjuvant radiotherapy presents no survival advantage though better local tumor control is obtained.  The use of hemopoietic stem cell transplantation (HSCT) as initial therapy remains controversial because of the overall excellent prognosis of children with advanced and unfavorable Hodgkin lymphoma to chemotherapy alone or in combination with radiotherapy.  At present HSCT should be reserved for patients after relapse or for those who are refractory to conventional therapy  ABVD regimen is typically reserved for the children with relapsed HL in combination with Ifosfamide, Etoposide, and Prednisolone (IEP).  In addition to the chemotherapy, relapsed disease would require radiotherapy, and may require autologous stem cell transplant. ABVD Protocol for Treating Relapse of Hodgkin Lymphoma Drug Days Doses Doxorubicin ( Adriamycin) 1 & 15 25mg/m2, Iv slow Bleomycin 1 & 15 10 units/m2, Iv slow Vinblastine 1 & 15 6mg/ m2, Iv slow Dacarbazine ( DTIC) 1& 15 375mg/m2, Iv infusion over 1 hour 8 Nursing Management Nursing management for Hodgkin lymphoma requires a holistic approach, focusing on both the physical and emotional well-being of the patient throughout the entire care continuum.\ Nursing management for Hodgkin lymphoma involves comprehensive care to support patients throughout the diagnosis, treatment, and recovery phases. Here are key aspects of nursing management for Hodgkin lymphoma: Assessment and Monitoring:  Regularly assess the patient's physical and emotional well-being.  Monitor vital signs and laboratory values, including complete blood counts (CBC) and liver function tests.  Evaluate the patient for signs of infection, bleeding, or other complications.  Offer emotional support and address any psychological concerns the patient may have.  Facilitate support groups or counseling services to help patients cope with the emotional impact of the diagnosis and treatment. Improving breathing pattern  Evaluate and track the patient’s breathing rate, saturation. Dyspnea and the use of auxiliary muscles, nasal flaring, should be reported  Monitor and analyze skin color, taking note of any pallor or cyanosis (particularly in nail beds, ear lobes, and lips).  Place the patient in a comfortable position, such as elevated head of bed or sitting upright leaning forward.  Periodically reposition to mobilize secretion.  Rest periods should be scheduled in between activities.  Identify and promote energy-saving methods ( rest periods before and after meals, , sitting for care).  Maintain a calm and quiet environment to relax the body, saves energy, and reduces oxygen consumption. 9   Administer nebulization.  Supplemental oxygen should be provided. Pain Management:  Assess and manage pain effectively, considering both pharmacological and non- pharmacological interventions.  Use distraction techniques such as deep breathing, listening to music, watching Tv, Playing games.  Administer analgesics  Use heat and cold application over affected areas.  Administer narcotic analgesics. Maintaining Temperature  Monitor temperature  Remove extra clothes  Tepid sponging should be done  Maintain ventilation  Maintain hydration  Administer Paracetamol Restoring Healthy Oral Mucosa  Frequently assess oral cavity for redness, lesions, ulcers, laques, or bleeding to provide baseline data.  Offer ice chips frequently while child is NPO to maintain hydration of mucosa.  Use only a soft toothbrush for dental care avoiding excessive pressure with brushing, Promoting Body Image  Acknowledge the child's feelings of anger over body changes and illness: venting feelings is associated with less body image disturbance. 10   Encourage the child or teen to choose a wig or hats and scarves to involve the child in making decisions about appearance.  Support the child's or teen's choices of comfortable, fashionable clothing to disguise weight loss or scarring while promoting self-esteem.  Involve the child in the decision-making process, as a sense of control will improve body image. Encourage the child to spend time with peers who have experienced hair limb, or weight loss. ( self help group) Infection Prevention:  Educate patients on the importance of infection prevention measures, especially during immunosuppressive treatments.  Monitor for signs of infection and implement appropriate interventions promptly.  Administer antibiotics for temperature greater than 38.4°C to decrease likelihood of overwhelming sepsis  Maintain meticulous hand hygiene (including family, visitors, staffs) to minimize spread of infectious organisms.  Maintain isolation as prescribed to minimize exposure to infectious organisms.  Use of face mask, hand sanitizer.  Avoid rectal temperatures and examinations, intramuscular injections, and urinary catheterization when child is neutropenia to decrease possibility of introducing microorganisms.  Educate family and visitors that child should be restricted from contact with known infectious exposures (in hospital and at home).  Promote nutrition and appropriate rest to maximize body's potential to heal. Alleviating Nausea and Vomiting  Administer antiemetic prior to chemotherapy and as needed thereafter to decrease frequency of nausea. 11   Assess frequency of vomiting and level of hydration to provide baseline data and recognize alterations early.  Offer frequent, smaller meals or snacks: smaller amounts are less likely to be vomited.  Avoid spicy foods to avoid stomach upset  Allow bubbles to dissipate from carbonated beverages before they are ingested: carbonation may contribute nausea. Promoting Adequate Nutrition  Determine body weight and length/height norm age or find out what the child's pretreatment measurements were to determine goal to work toward  Determine child's food preferences and provide favorite foods as able to increase the likelihood that the child will consume adequate amounts of foods  Administer antiemetics.  Weigh child daily or weekly) and measure length height weekly to monitor for growth.  Offer highest-calorie meals at the time of day when the child has good appetite  Administer vitamin and mineral supplements as prescribed to attain/maintain vitamin and mineral balance in the body.  Administer total parenteral infusion and intravenous lipids as ordered to provide sequate nutrition for healing. Promoting Child and Family Coping  Provide emotional support to the child and family: improves coping abilities.  Actively listen to the child's and family's concerns: validates their feelings and establishes trust.  Provide clear information according to their understanding level.  Provide open communication with the child and siblings: children appreciate honesty about their illness and coping is improved.  Encourage to have positive thoughts, listen to Bhajans, listening motivational speech remember god.  The parents current knowledge and their level of understanding were assessed.  Encourage the parent and child to ask question when in doubt. 12   The instruction and sessions were kept short.  Give terminally ill children permission to discuss their feelings about their illness, allowing them to conquer fears and express love for their family and friends.  Encourage families to be honest with siblings about the treatment and prognosis of the child with cancer: children often sense what is going on and cope better when they are prepared and are given an honest explanation of events. Prognosis  Worldwide outcome for this disease is 80-90%. Relapsed lymphoblastic lymphoma can be treated with chemotherapy and stem cell transplant, but the outcomes are poor.  Overall outcome of mature B-cell neoplasms in children is 80%, with outcome of >95% in early stages.  Relapses occur early and are very aggressive. Despite attempts to treat relapsed disease with high dose chemotherapy and autologous stem cell transplant, the overall outcome is good. Complications  Infections  Pancytopenia  Secondary malignancies 13 14