Hodgkin's lymphoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2], Sowminya Arikapudi, M.B,B.S. [3]
Overview
Hodgkin's lymphoma was first discovered by Thomas Hodgkin, a British physician, in 1832. Hodgkin's lymphoma is a type of lymphoma, in which cancer originates from a specific type of white blood cells called lymphocytes. Hodgkin's lymphoma may be classified according to World Health Organization (WHO) into 2 subtypes: nodular lymphocyte predominance and classical Hodgkin lymphoma. On gross pathology, white-grey, uniform, and enlarged lymphnodes are characteristic findings of Hodgkin's lymphoma. On microscopic histopathological analysis, Reed-Sternberg cells, reactive cell infiltrate, and complete or partial effacement of the lymph node architecture are characteristic findings of Hodgkin's lymphoma. There are no established causes for Hodgkin's lymphoma. Hodgkin's lymphoma must be differentiated from sarcoidosis, lymphocytic lymphoma, miliary tuberculosis, infectious mononucleosis, thoracic aortic aneurysm, substernal goiter, thymoma, actinomycosis, chronic lymphocytic leukemia, superior vena cava syndrome, unicentric castleman disease, adult still disease, small cell lung carcinoma, and malignant histiocytosis. Hodgkin's lymphoma has a bimodal age distribution that differs geographically and ethnically in industrialized countries; the early peak occurs in the middle-to-late 20s and the second peak after age 50 years. In developing countries, the early peak occurs before adolescence.[1]In 2015, the incidence of Hodgkins lymphoma was estimated to be 3 cases per 100,000 individuals in the United States.[1]The incidence of Hodgkin's lymphoma is higher among patients with HIV/AIDS; however, in contrast to many other lymphomas associated with HIV infection, Hodgkin's lymphoma occurs most commonly in patients who do not have severe immunosupression.[2]The risk factors in the development of Hodgkin's lymphoma are Epstein-Barr virus, family history, HIV infection. Other possible risk factors include genetics, infectious mononucleosis, autoimmune diseases, immunodeficiency, tobacco, and socio-economic status and family features. Screening for Hodgkin's lymphoma is not recommended. The early stage of the Hodgkin's lymphoma is associated with the most favorable prognosis. The 5-year survival rate of patients with Hodgkin's lymphoma varies with the stage of the disease. According to the Lugano classification and Cotswold's modification of the original Ann arbor staging system, there are four stages of Hodgkin's lymphoma based on the number of nodes and extranodal involvement. The most common symptoms of Hodgkin's lymphoma include fatigue, fever and chills, itching, loss of appetite, soaking night sweats, weight loss, and painless swelling of the lymph nodes in the neck, axilla, or groin (swollen glands). Less common symptoms of Hodgkin's lymphoma include coughing, chest pain, or breathing problems if there are swollen lymph nodes in the chest, pain or feeling of fullness below the ribs due to swollen spleen or liver, pain in lymph nodes after drinking alcohol, skin blushing or flushing, bone pain, and leg swelling. Common physical examination findings of Hodgkin's lymphoma include fever, pruritus, petechiae, jaundice, chest tenderness, wheeze, superior vena cava syndrome, abdominal tenderness, hepatomegaly, splenomegaly, fracture, peripheral lymphadenopathy, and central lymphadenopathy. Laboratory tests for Hodgkin's lymphoma include complete blood count (CBC), blood chemistry studies, HIV blood test, immunohistochemistry, erythrocyte sedimentation rate, and immunophenotyping. Chest, spine, pelvic, and long bone x rays may be helpful in the diagnosis of Hodgkin's lymphoma. Chest, abdomen, and pelvis CT and MRI scans may be helpful in the diagnosis of Hodgkin's lymphoma. On ultrasound, Hodgkin's lymphoma is characterized by hepatomegaly and splenomegaly. Lymph node or extranodal tissue biopsy is diagnostic of Hodgkin's lymphoma. Chest, abdomen, and pelvis PET scans may be helpful in the diagnosis of Hodgkin's lymphoma. In some cases a Gallium Scan may be used instead of a PET scan. Other diagnostic studies for the diagnosis of Hodgkin's lymphoma include bone marrow biopsy, bone scan, lymphangiogram, or laparotomy. The optimal therapy for Hodgkin's lymphoma depends on the stage at diagnosis, age, type, and size of tumor. The predominant therapy for Hodgkin's lymphoma is chemotherapy. Adjunctive radiation therapy and stem cell transplant may be required. Surgical intervention is not recommended for the management of Hodgkin's lymphoma.
Historical perspective
Hodgkin's lymphoma was first discovered by Thomas Hodgkin, a British physician, in 1832.
Classification
Hodgkin's lymphoma may be classified according to World Health Organization (WHO) into 2 subtypes: nodular lymphocyte predominance and classical Hodgkin lymphoma.
Pathophysiology
On gross pathology, white-grey, uniform, and enlarged lymphnodes are characteristic findings of Hodgkin's lymphoma. On microscopic histopathological analysis, Reed-Sternberg cells, reactive cell infiltrate, and complete or partial effacement of the lymph node architecture are characteristic findings of Hodgkin's lymphoma.
Causes
There are no established causes for Hodgkin's lymphoma.
Differential diagnosis
Hodgkin's lymphoma must be differentiated from sarcoidosis, lymphocytic lymphoma, miliary tuberculosis, infectious mononucleosis, thoracic aortic aneurysm, substernal goiter, thymoma, actinomycosis, chronic lymphocytic leukemia, superior vena cava syndrome, unicentric castleman disease, adult still disease, small cell lung carcinoma, and malignant histiocytosis.
Epidemiology and Demographics
Hodgkin's lymphoma has a bimodal age distribution that differs geographically and ethnically in industrialized countries; the early peak occurs in the middle-to-late 20s and the second peak after age 50 years. In developing countries, the early peak occurs before adolescence.[1]In 2015, the incidence of Hodgkins lymphoma was estimated to be 3 cases per 100,000 individuals in the United States.[1]The incidence of Hodgkin's lymphoma is higher among patients with HIV/AIDS; however, in contrast to many other lymphomas associated with HIV infection, Hodgkin's lymphoma occurs most commonly in patients who do not have severe immunosupression.[3]
Risk Factors
The risk factors in the development of Hodgkin's lymphoma are Epstein-Barr virus, family history, HIV infection. Other possible risk factors include genetics, infectious mononucleosis, autoimmune diseases, immunodeficiency, tobacco, and socio-economic status and family features.
Screening
Screening for Hodgkin's lymphoma is not recommended.
Prognosis
The early stage of the Hodgkin's lymphoma is associated with the most favorable prognosis. The 5-year survival rate of patients with Hodgkin's lymphoma varies with the stage of the disease.
Diagnosis
Staging
According to the Lugano classification and Cotswold's modification of the original Ann arbor staging system, there are four stages of Hodgkin's lymphoma based on the number of nodes and extranodal involvement.
Symptoms
The most common symptoms of Hodgkin's lymphoma include fatigue, fever and chills, itching, loss of appetite, soaking night sweats, weight loss, and painless swelling of the lymph nodes in the neck, axilla, or groin (swollen glands). Less common symptoms of Hodgkin's lymphoma include coughing, chest pain, or breathing problems if there are swollen lymph nodes in the chest, pain or feeling of fullness below the ribs due to swollen spleen or liver, pain in lymph nodes after drinking alcohol, skin blushing or flushing, bone pain, and leg swelling.
Physical Examination
Common physical examination findings of Hodgkin's lymphoma include fever, pruritus, petechiae, jaundice, chest tenderness, wheeze, superior vena cava syndrome, abdominal tenderness, hepatomegaly, splenomegaly, fracture, peripheral lymphadenopathy, and central lymphadenopathy.
Laboratory Findings
Laboratory tests for Hodgkin's lymphoma include complete blood count (CBC), blood chemistry studies, HIV blood test, immunohistochemistry, erythrocyte sedimentation rate, and immunophenotyping.
X Ray Findings
Chest, spine, pelvic, and long bone x rays may be helpful in the diagnosis of Hodgkin's lymphoma.
CT
Chest, abdomen, and pelvis CT scans may be helpful in the diagnosis of Hodgkin's lymphoma.
MRI
Chest, abdomen, and pelvic MRI scans may be helpful in the diagnosis of Hodgkin's lymphoma.
Ultrasound
On ultrasound, Hodgkin's lymphoma is characterized by hepatomegaly and splenomegaly.
Biopsy
Lymph node or extranodal tissue biopsy is diagnostic of Hodgkin's lymphoma.
Other Imaging Findings
Chest, abdomen, and pelvis PET scans may be helpful in the diagnosis of Hodgkin's lymphoma. In some cases a Gallium Scan may be used instead of a PET scan.
Other Diagnostic Studies
Other diagnostic studies for the diagnosis of Hodgkin's lymphoma include bone marrow biopsy, bone scan, lymphangiogram, or laparotomy.
Treatment
Medical Therapy
The optimal therapy for Hodgkin's lymphoma depends on the stage at diagnosis, age, type, and size of tumor. The predominant therapy for Hodgkin's lymphoma is chemotherapy. Adjunctive radiation therapy and stem cell transplant may be required.
Surgery
Surgical intervention is not recommended for the management of Hodgkin's lymphoma.
References
- ↑ 1.0 1.1 1.2 1.3 "National Caner Institute Childhood Hodgkin Lymphoma Treatment".
- ↑ Biggar RJ, Jaffe ES, Goedert JJ, Chaturvedi A, Pfeiffer R, Engels EA (2006). "Hodgkin lymphoma and immunodeficiency in persons with HIV/AIDS". Blood. 108 (12): 3786–91. doi:10.1182/blood-2006-05-024109. PMID 16917006.
- ↑ Biggar RJ, Jaffe ES, Goedert JJ, Chaturvedi A, Pfeiffer R, Engels EA (2006). "Hodgkin lymphoma and immunodeficiency in persons with HIV/AIDS". Blood. 108 (12): 3786–91. doi:10.1182/blood-2006-05-024109. PMID 16917006.