Medical Nursing (III)
Subtopic:
Hodgkin's disease
Hodgkin’s disease, also known as Hodgkin’s lymphoma, is a specific type of lymphoma.
LYMPHOMAS
Lymphomas are cancers that originate in lymphoid tissue. Lymphoma is a cancer affecting the immune system, specifically the immune cells called lymphocytes, which are a type of white blood cell. In lymphoma, there is an abnormal increase in the number of lymphatic cells, which can lead to enlargement of the liver (hepatomegaly), spleen (splenomegaly), and lymph nodes (lymphadenitis).
Classification of lymphomas:
Hodgkin’s lymphoma
Non-Hodgkin’s lymphoma
Hodgkin’s Lymphoma
Hodgkin’s lymphoma is characterized by the malignant proliferation of lymphoid cells that, upon microscopic examination (histology), show the presence of distinctive Reed-Sternberg cells.
Hodgkin’s Disease:
Hodgkin’s lymphoma is a malignant condition where the lymph glands become enlarged, and there is an increase in lymphoid tissue within the liver, spleen, and bone marrow. If left untreated, this disease can be fatal. It was first described in 1832 by the British physician Thomas Hodgkin.
The exact cause of Hodgkin’s lymphoma is not known, but it has been linked to the Epstein-Barr Virus (EBV). Exposure to and infection with EBV have been associated with its development, particularly in individuals under 15 years of age and over 50 years of age.
Hodgkin’s lymphoma can occur in individuals of any sex and age, but it is more commonly found in males and has a bimodal age distribution, with peaks in incidence between 20-35 years and 50-70 years.
The disease involves the abnormal and uncontrolled growth (neoplastic proliferation) of an atypical type of lymphoid cell, specifically the Reed-Sternberg cell. It is primarily a disease of B-cells (a type of lymphocyte).
Clinical staging of Hodgkin’s lymphoma:
Stage I: Involvement of a single lymph node region or a single extranodal site (outside the lymph nodes).
Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (the muscle separating the chest and abdomen), or localized involvement of an extranodal site and one or more lymph node regions on the same side of the diaphragm.
Stage III: Involvement of lymph node regions on both sides of the diaphragm.
Stage III(1): Lymphatic involvement of the upper abdomen, specifically the spleen, celiac, and portal lymph nodes.
Stage III(2): Lymphatic involvement of the upper abdomen (spleen, celiac, and portal nodes) and the lower abdominal nodes in the periaortic, mesenteric, and iliac regions.
Stage IV: Diffuse or widespread dissemination of the disease involving one or more extralymphatic organs or tissues, with or without lymph node involvement. These can be indicated by the following codes:
H: Hepatic (liver involvement)
L: Lung (lung involvement)
P: Pleura (lining of the lungs)
M: Marrow (bone marrow involvement)
D: Dermal (skin involvement)
O: Osseous (bone involvement)
WHO Classification:
Nodular sclerosing HL: The most common subtype of Hodgkin’s lymphoma, characterized by large tumor nodules.
Mixed cellularity subtype HL: Another common subtype.
Lymphocyte-rich: A rare subtype.
Lymphocyte-depleted: A rare subtype.
Clinical features of Hodgkin’s lymphoma:
The most common presentation is the enlargement of one lymph node or a group of lymph nodes. This may be discovered during investigations for nonspecific symptoms such as unexplained weight loss (more than 10% in 6 months), fever, and persistent itching (pruritus).
Enlarged lymph nodes in Hodgkin’s lymphoma are typically replaced by a firm, rubbery tissue that feels elastic upon palpation and has a pinkish-white color. If untreated, the disease can spread to involve the spleen, liver, and bone marrow. The enlargement of lymph nodes usually develops gradually and without causing significant pain. The affected nodes feel painless and have a rubbery consistency when touched.
In some cases, systemic symptoms such as fever, weight loss, weakness, night sweats, and anemia may be present. Patients may experience coughing and shortness of breath (dyspnea) due to enlarged lymph nodes in the hilum (the region where blood vessels, bronchi, and nerves enter the lung) or thoracic (chest) area. Other signs and symptoms can include chills, a rapid heart rate (tachycardia), and enlargement of the liver and spleen (hepatosplenomegaly). Abdominal distention and the accumulation of fluid in the abdominal cavity (ascites) can occur due to enlarged abdominal lymph nodes, particularly the retroperitoneal nodes located behind the abdominal cavity.
A peculiar symptom reported by some patients is pain in the affected lymph nodes after consuming alcohol; the underlying cause of this phenomenon is not well understood. If the lymph nodes in the mediastinum (the space between the lungs) are involved, it can lead to difficulty swallowing (dysphagia). Jaundice, or yellowing of the skin and eyes, can occur if the liver is involved. Bone involvement can cause bone pain, and in cases where the spinal cord is compressed due to extradural (outside the dura mater, the outermost membrane covering the spinal cord) involvement, it can result in paraplegia (paralysis of the lower half of the body). Palpable masses may be felt in the abdomen. In the advanced stages of the disease, anemia can develop due to decreased production of red blood cells (erythropoiesis) and increased destruction of red blood cells (hemolysis).
Differential Diagnosis:
It is important to differentiate Hodgkin’s lymphoma from other conditions that can cause similar symptoms, including:
Non-Hodgkin’s lymphoma
Lymphadenitis (inflammation of the lymph nodes) secondary to tuberculosis (TB) and cat scratch disease
Pseudolymphoma, a condition sometimes caused by the drug phenytoin
Lymphomatoid granulomatosis, a rare disorder characterized by blood vessel inflammation
Sarcoidosis, a disease involving abnormal collections of inflammatory cells
HIV disease
Systemic lupus erythematosus (SLE), an autoimmune disease
Investigations Done in Hodgkin’s Lymphoma:
Several investigations are crucial in diagnosing and staging Hodgkin’s lymphoma:
Lymph node biopsy: Microscopic examination of an affected lymph node to confirm the diagnosis and identify Reed-Sternberg cells.
Bone marrow examination: Evaluation of the bone marrow to determine if the lymphoma has spread.
Peripheral blood film: A blood test that may reveal normochromic normocytic anemia (red blood cells are normal in size and hemoglobin content), eosinophilia (increased eosinophils, a type of white blood cell), neutrophilia (increased neutrophils, another type of white blood cell), and lymphopenia (decreased lymphocytes).
Raised Erythrocyte Sedimentation Rate (ESR): An elevated ESR is a nonspecific marker of inflammation.
Chest X-ray: Can reveal a mediastinal mass, indicating enlarged lymph nodes in the chest.
CT scan of the chest, abdomen, and pelvis: Used to define the extent and stage of the disease.
Lymphangiography: An imaging technique to visualize the lymphatic system.
It’s worth noting that in tuberculous lymphadenitis, the lymph nodes are typically large, tender, and firm, and they may rupture to form sinuses (abnormal channels).
Management of Hodgkin’s Lymphoma:
The treatment approach for Hodgkin’s lymphoma depends on the stage and extent of the disease:
Radiation therapy: Primarily used for localized disease.
Short course combination therapy with less extensive radiation: May be used for certain stages of localized disease.
Radiation combined with chemotherapy: The standard treatment for disseminated (widespread) disease.
Cytotoxic drugs combined with steroids: Chemotherapy regimens commonly used include:
MOPP: A combination of Mustine (nitrogen mustard), Oncovin (vincristine), Procarbazine, and Prednisone, administered on specific days of the treatment cycle.
ABVD: A combination of Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine, also administered on specific days of the treatment cycle.
Nursing Care:
Nursing care for patients with Hodgkin’s lymphoma focuses on managing the side effects of treatment, particularly pancytopenia (a deficiency of red blood cells, white blood cells, and platelets) and other drug effects. This includes:
Psychological support: Providing emotional and mental support to help patients cope with their diagnosis and treatment.
Nutritional support: Ensuring patients receive adequate nutrition to maintain strength and manage treatment side effects.
Regular hygiene: Maintaining strict hygiene practices to prevent infections, as patients are often immunocompromised.
NON-HODGKIN’S (NON-BURKITT’S) LYMPHOMA (NHL)
Non-Hodgkin’s lymphoma is a type of cancer that originates in the lymphatic system. The cancer begins in the cells of the immune system, and while it can start in various parts of the lymphatic system, it is most commonly found in the lymph nodes. In NHL, lymphocytes, predominantly B-cells, become abnormal. However, T-cells can also be affected.
These abnormal cells replicate and divide continuously, unlike normal cells. These cancerous cells also have an extended lifespan and lose their ability to defend the body against infections. The excess cells often accumulate and form a mass known as a tumor. There are more than 60 specific subtypes of non-Hodgkin’s lymphoma
Staging of Non-Hodgkin Lymphoma:
The stage of non-Hodgkin lymphoma (NHL) is determined by the number of affected areas and imaging results:
Stage I: Lymphoma cells are confined to a single lymph node group (e.g., neck or underarm). Alternatively, if the abnormal cells are not in the lymph nodes, they are present in only one part of a tissue or organ (excluding the liver or bone marrow).
Stage II: Lymphoma cells are found in at least two lymph node groups on the same side of the diaphragm (either above or below it). Alternatively, the lymphoma cells are in one part of an organ and the nearby lymph nodes on the same side of the diaphragm. There may also be lymphoma cells in other lymph node groups on the same side of the diaphragm.
Stage III: Lymphoma is present in lymph nodes both above and below the diaphragm. It may also be found in one part of a tissue or organ near these lymph node groups.
Stage IV: Lymphoma cells are found in multiple parts of one or more organs or tissues, in addition to lymph nodes. It may also involve the liver, blood, or bone marrow.
Recurrent: The disease returns after treatment.
In addition to the numerical stages, the disease may be further classified as stage A or B:
A: Absence of significant symptoms like unexplained weight loss, drenching night sweats, or persistent fevers.
B: Presence of significant symptoms such as unexplained weight loss, drenching night sweats, or persistent fevers.
Risk Factors for Non-Hodgkin’s Lymphoma:
Weakened Immune System: A compromised immune system, whether due to inherited conditions or medications used after organ transplantation, increases the risk of lymphoma. Certain infections are also associated with a higher risk:
Human Immunodeficiency Virus (HIV): Individuals with HIV have a significantly increased risk of developing certain types of NHL.
Epstein-Barr Virus (EBV): EBV infection is linked to Burkitt lymphoma.
Helicobacter pylori: Infection with H. pylori, bacteria that can cause stomach ulcers, increases the risk of lymphoma in the stomach lining.
Human T-cell Leukemia/Lymphoma Virus Type 1 (HTLV-1): Infection with HTLV-1 elevates the risk of lymphoma.
Hepatitis C Virus: Some studies suggest a link between hepatitis C infection and an increased risk of lymphoma, but further research is needed.
Age: While NHL can occur in younger individuals, the risk increases with age, with most diagnoses occurring in people over 60.
Obesity: Obesity is considered a possible risk factor for NHL.
Occupation: Exposure to herbicides or certain other chemicals in the workplace may increase the risk.
Clinical Features of Non-Hodgkin’s Lymphoma:
NHL is uncommon before the age of 40. While swollen lymph nodes (lymphadenopathy) are a common sign, NHL often spreads to areas outside the lymph nodes early in the disease. Therefore, the first presentation might be in the skin, gastrointestinal tract, central nervous system (CNS), or lungs. Although often initially without symptoms, systemic symptoms similar to Hodgkin’s lymphoma can develop. Bone marrow involvement can lead to pancytopenia (deficiency of blood cells). Infection is a common complication.
Common clinical features include:
Progressive, painless enlargement of lymph nodes
Unexplained weight loss
Night sweats
Persistent fever
Anemia
Persistent weakness and fatigue
Enlargement of the liver (hepatomegaly)
Abdominal pain, swelling, and a feeling of fullness
Enlargement of the spleen (splenomegaly)
Diagnosis and Investigation:
Diagnosis of NHL involves several steps:
Physical Exam: Checking for swollen lymph nodes in the neck, underarms, and groin, as well as examining the spleen and liver for enlargement.
Blood Tests: A complete blood count (CBC) is performed to assess the number of white blood cells and other blood components. Levels of substances like lactate dehydrogenase (LDH), which may be elevated in lymphoma, are also checked.
Chest X-rays: Used to detect swollen lymph nodes or other signs of disease in the chest.
Biopsy: A tissue sample, ideally an entire lymph node, is removed and examined under a microscope by a pathologist to confirm the presence of lymphoma cells.
Bone Marrow Biopsy: A small sample of bone and bone marrow is extracted, typically from the hipbone or breastbone, and examined for lymphoma cells. Local anesthesia is used to minimize discomfort.
Liver Function Tests (LFTs) and Renal Function Tests (RFTs): To assess the health of the liver and kidneys.
Serology Tests for HIV: To determine HIV status, as HIV is a risk factor for NHL.
CT Scan: Detailed images of the head, neck, chest, abdomen, or pelvis are obtained using X-rays and computer technology. Contrast material may be injected or ingested to enhance visualization of lymph nodes and abnormal areas.
MRI: Powerful magnets linked to a computer create detailed images of tissues, particularly useful for examining the spinal cord, bone marrow, or brain.
Ultrasound: Sound waves are used to create images of internal organs and tissues, helping to identify potential tumors based on their echo patterns.
Spinal Tap (Lumbar Puncture): Cerebrospinal fluid (CSF) is collected and examined for lymphoma cells or other abnormalities.
PET Scan: A small amount of radioactive sugar is injected, and a machine detects areas of high metabolic activity, as lymphoma cells utilize sugar faster than normal cells, appearing brighter on the scan.
The stage of NHL is determined based on the location of lymphoma cells in the lymph nodes and other organs or tissues.
Management of Non-Hodgkin’s Disease:
Treatment for NHL is tailored to the individual patient and depends on several factors, including the specific type of NHL, the stage of the lymphoma, the growth rate of the cancer (indolent or aggressive), the patient’s age, and other health conditions. Specialists involved in the treatment of NHL include hematologists, medical oncologists, radiation oncologists, oncology nurses, and registered dietitians.
Watchful Waiting: For patients with indolent (slow-growing) NHL without significant symptoms, immediate treatment may not be necessary. Instead, the healthcare team closely monitors the patient’s condition, and treatment is initiated if symptoms develop. Chemotherapy and biological therapy are typically used when indolent lymphoma becomes symptomatic. Radiation therapy may be used for Stage I or Stage II lymphoma.
Chemotherapy: Uses drugs to kill cancer cells throughout the body. These drugs can be administered orally, intravenously, or directly into the spinal fluid, depending on the stage and nature of the cancer and the specific drugs used. Initial treatment may involve drugs like cyclophosphamide and chlorambucil. For recurrent NHL, combinations like CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or CVP (cyclophosphamide, vincristine, and prednisone) may be used. Side effects can include loss of appetite, nausea and vomiting, diarrhea, difficulty swallowing, mouth sores, hair loss, increased risk of infections, easy bruising or bleeding, skin rashes, headaches, weakness, and fatigue.
Biological Therapy: This treatment enhances the body’s immune system to fight cancer. Monoclonal antibodies, such as interferon, interleukin-2, and tumor necrosis factor (proteins created in a lab that can bind to cancer cells and help destroy them), are administered intravenously. Side effects may include flu-like symptoms such as fever, chills, headache, weakness, and nausea, which are usually manageable. Rarely, more serious side effects like breathing problems, low blood pressure, or severe skin rashes can occur.
Radiation Therapy (Radiotherapy): Uses high-energy rays to kill lymphoma cells and shrink tumors. There are two main types:
External Radiation: A machine directs radiation beams at the affected area. This is a local therapy, affecting only the cells in the treated region. Treatments are typically given daily, five days a week, for several weeks. Side effects to the abdomen can include nausea, vomiting, and diarrhea. Treatment to the chest and neck may cause a dry, sore throat and difficulty swallowing. The skin in the treated area may become red, dry, and tender.
Systemic Radiation: Involves injecting radioactive material that travels throughout the body, often attached to monoclonal antibodies that target lymphoma cells. This radiation destroys lymphoma cells throughout the body. Side effects can include fatigue, increased risk of infections, and worsening of the side effects seen with external radiation.
Stem Cell Transplantation: Considered if lymphoma returns after initial treatment. This procedure allows patients to receive high doses of chemotherapy and/or radiation therapy, which destroy both lymphoma cells and healthy blood cells in the bone marrow. Following the high-dose treatment, healthy stem cells are transplanted back into the patient’s bloodstream through a central line. These new stem cells help the body produce new, healthy blood cells. The stem cells can be harvested from the patient’s own body (autologous transplant), from a related donor such as a sibling or parent (allogeneic transplant), or from an identical twin (syngeneic transplant).
Supportive Care: Aims to manage symptoms, alleviate treatment side effects, and provide emotional and physical support. This includes:
Nutrition: Ensuring adequate calorie and protein intake to maintain weight and strength.
Activity: Encouraging gentle exercise like walking or swimming to maintain strength, reduce nausea and pain, and manage stress.
Follow-Up Care: Regular checkups after treatment to monitor recovery and check for recurrence. These checkups may include physical exams, lab tests, and imaging.
Social Support: Providing access to resources and support groups, including doctors, nurses, social workers, and other patients and families, to help cope with the diagnosis and treatment.
Treating Treatment Side Effects: Managing side effects as they arise.
Treating Helicobacter pylori: If present, H. pylori infection is treated with antibiotics.
Surgical Intervention: Surgery may be needed to correct strictures or obstructions caused by the lymphoma.
Encouraging Bladder Training, Habit Retraining, and Fluid Intake: To manage any bladder issues.