AIDS-related lymphoma describes

lymphoma Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). The name typically refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlarged lymph node ...

s occurring in

patient A patient is any recipient of health care services that are performed by Health professional, healthcare professionals. The patient is most often Disease, ill or Major trauma, injured and in need of therapy, treatment by a physician, nurse, op ...

s with acquired immunodeficiency syndrome (

AIDS The HIV, human immunodeficiency virus (HIV) is a retrovirus that attacks the immune system. Without treatment, it can lead to a spectrum of conditions including acquired immunodeficiency syndrome (AIDS). It is a Preventive healthcare, pr ...

). A lymphoma is a type of cancer arising from

lymphoid The lymphatic system, or lymphoid system, is an organ system in vertebrates that is part of the immune system and complementary to the circulatory system. It consists of a large network of lymphatic vessels, lymph nodes, lymphoid organs, lympha ...

cells Cell most often refers to: * Cell (biology), the functional basic unit of life * Cellphone, a phone connected to a cellular network * Clandestine cell, a penetration-resistant form of a secret or outlawed organization * Electrochemical cell, a d ...

. In AIDS, the incidence of

non-Hodgkin's lymphoma Non-Hodgkin lymphoma (NHL), also known as non-Hodgkin's lymphoma, is a group of blood cancers that includes all types of lymphomas except Hodgkin lymphomas. Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tire ...

, primary

cerebral Cerebral may refer to: * Of or relating to the brain * Cerebrum, the largest and uppermost part of the brain * Cerebral cortex, the outer layer of the cerebrum * Retroflex consonant, also referred to as a cerebral consonant, a type of consonant so ...

lymphoma and

Hodgkin's disease Hodgkin lymphoma (HL) is a type of lymphoma in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells (RS cells) are present in the lymph nodes. The condition was named a ...

are all increased. There are three different varieties of AIDS-related lymphoma:

Diffuse large B-cell lymphoma Diffuse large B-cell lymphoma (DLBCL) is a cancer of B cells, a type of lymphocyte that is responsible for producing antibodies. It is the most common form of non-Hodgkin lymphoma among adults, with an annual incidence of 7–8 cases per 100,000 ...

, B-cell immunoblastic lymphoma, and

Burkitt's lymphoma Burkitt's lymphoma is a cancer of the lymphatic system, particularly B lymphocytes found in the germinal center. It is named after Denis Parsons Burkitt, the Irish surgeon who first described the disease in 1958 while working in equatorial Africa ...

(small non-cleaved cell lymphoma).

Signs and symptoms

The histologic classification of the

, as well as the locations and severity of the disease, all influence the clinical presentation of HIV-related

lymphomas Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). The name typically refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlarged lymph node ...

. HIV-related

are more likely to present with advanced stage disease, constitutional symptoms (also known as "B" symptoms;

fever Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...

weight loss Weight loss, in the context of medicine, health, or physical fitness, refers to a reduction of the total body mass, by a mean loss of fluid, body fat (adipose tissue), or lean mass (namely bone mineral deposits, muscle, tendon, and other conn ...

, and

night sweats Night sweats or nocturnal hyperhydrosis is the repeated occurrence of excessive sweating during sleep. The person may or may not also perspire excessively while awake. One of the most common causes of night sweats in women over 40 is the horm ...

), extranodal involvement, or disease involving unusual locations (such as the body cavity or soft tissue) than

in the

HIV The human immunodeficiency viruses (HIV) are two species of '' Lentivirus'' (a subgroup of retrovirus) that infect humans. Over time, they cause acquired immunodeficiency syndrome (AIDS), a condition in which progressive failure of the im ...

-negative population. HIV-related lymphomas can present with a variety of clinical symptoms, including

organomegaly Organomegaly is the abnormal enlargement of organs. For example, cardiomegaly is enlargement of the heart. Visceromegaly is the enlargement of abdominal organs. Examples of visceromegaly are enlarged liver (hepatomegaly), spleen (splenomegaly Sp ...

lymphadenopathy Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In c ...

, and/or constitutional symptoms. Unknown

cytopenias Cytopenia is a reduction in the number of mature blood cells. It can have many causes, and commonly occurs in people with cancer being treated with radiation therapy or chemotherapy. Types * Anemia – a reduction of the red blood cells in the bo ...

tumor lysis syndrome Tumor lysis syndrome (TLS) is a group of metabolic abnormalities that can occur as a complication from the treatment of cancer, where large amounts of tumor cells are killed off ( lysed) from the treatment, releasing their contents into the blood ...

(including

lactic acidosis Lactic acidosis refers to the process leading to the production of lactate by anaerobic metabolism. It increases hydrogen ion concentration tending to the state of acidemia or low pH. The result can be detected with high levels of lactate and lo ...

hyperkalemia Hyperkalemia is an elevated level of potassium (K+) in the blood. Normal potassium levels are between 3.5 and 5.0 mmol/L (3.5 and 5.0 mEq/L) with levels above 5.5mmol/L defined as hyperkalemia. Typically hyperkalemia does not cause symptoms. Oc ...

hyperuricemia Hyperuricaemia or hyperuricemia is an abnormally high level of uric acid in the blood. In the pH conditions of body fluid, uric acid exists largely as urate, the ion form. Serum uric acid concentrations greater than 6 mg/dL for females, 7 ...

hypocalcemia Hypocalcemia is a medical condition characterized by low calcium levels in the blood serum. The normal range of blood calcium is typically between 2.1–2.6 mmol/L (8.8–10.7 mg/dL, 4.3–5.2 mEq/L), while levels less than 2.1 ...

hyperphosphatemia Hyperphosphatemia is an electrolyte disorder in which there is an elevated level of phosphate in the blood. Most people have no symptoms while others develop calcium deposits in the soft tissue. The disorder is often accompanied by low calci ...

, and elevated

lactate dehydrogenase Lactate dehydrogenase (LDH or LD) is an enzyme found in nearly all living cells. LDH catalyzes the conversion of pyruvic acid, pyruvate to lactic acid, lactate and back, as it converts NAD+ to NADH and back. A dehydrogenase is an enzyme that t ...

), and other isolated laboratory abnormalities (such as

hypercalcemia Hypercalcemia, also spelled hypercalcaemia, is a high calcium (Ca2+) level in the blood serum. The normal range for total calcium is 2.1–2.6 mmol/L (8.8–10.7 mg/dL, 4.3–5.2 mEq/L), with levels greater than 2.6 mmol/L def ...

) are observed in certain patients.

Causes

-positive individuals'

vary widely and can be classified into several histologic subtypes. The two primary

types that develop in

-positive individuals are

(NHL) and

Hodgkin's lymphoma Hodgkin lymphoma (HL) is a type of lymphoma in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells (RS cells) are present in the lymph nodes. The condition was named a ...

(HL).

is a highly aggressive type of

B-cell lymphoma The B-cell lymphomas are types of lymphoma affecting B cells. Lymphomas are Tumors of the hematopoietic and lymphoid tissues, "blood cancers" in the lymph nodes. They develop more frequently in older adults and in immunocompromised individuals. ...

. It is distinguished by the widespread proliferation of large neoplastic

B lymphocytes B cells, also known as B lymphocytes, are a type of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted or inserted into the plasm ...

with nuclei that are equal to or larger than normal histiocytic nuclei. The illness manifests with B symptoms at an advanced stage of the illness. It mostly affects patients who are severely immunosuppressed and can occur at nodal or extranodal sites, with the

gastrointestinal tract The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the Digestion, digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascula ...

being the most common site. It makes up between 45 and 50 percent of all

seen in this group, making it the most prevalent AIDS-associated lymphoma subtype. All ages are affected by DLBCL, which typically manifests as a rapidly growing

lymph node A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that includ ...

mass in the neck or abdomen. Up to 40% of patients have extranodal extramedullary disease, and about 30% of patients exhibit B symptoms. The second most prevalent NHL subtype that affects HIV-positive individuals with a comparatively high

CD4 In molecular biology, CD4 (cluster of differentiation 4) is a glycoprotein that serves as a co-receptor for the T-cell receptor (TCR). CD4 is found on the surface of immune cells such as helper T cells, monocytes, macrophages, and dendritic c ...

cell count is

. Patients typically have elevated

levels and poor performance status. The

central nervous system The central nervous system (CNS) is the part of the nervous system consisting primarily of the brain, spinal cord and retina. The CNS is so named because the brain integrates the received information and coordinates and influences the activity o ...

is involved in 8 to 28% of cases, with extranodal involvement occurring more frequently. It usually manifests at a younger age and with

cell counts greater than 200 cells/μL. It develops quickly and is a kind of tumor that starts from

B cells B cells, also known as B lymphocytes, are a type of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted or inserted into the plasm ...

. In addition, it is fatal if untreated.

is linked to a high incidence of oral cavity involvement and makes up 10-15% of AIDS-defining lymphomas. Three clinical subtypes of

have been identified: endemic, sporadic, and

immunodeficiency Immunodeficiency, also known as immunocompromise, is a state in which the immune system's ability to fight infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that aff ...

-related. Thirty to forty percent of AIDS-related NHL cases are

subtype, which is most prevalent in

HIV/AIDS The HIV, human immunodeficiency virus (HIV) is a retrovirus that attacks the immune system. Without treatment, it can lead to a spectrum of conditions including acquired immunodeficiency syndrome (AIDS). It is a Preventive healthcare, pr ...

patients.

Primary central nervous system lymphoma Primary central nervous system lymphoma (PCNSL), also termed primary diffuse large B-cell lymphoma of the central nervous system (DLBCL-CNS), is a primary intracranial tumor appearing mostly in patients with severe immunodeficiency (typically pat ...

(PCNSL) is a subtype of NHL that impacts the eyes, brain, spine, and

cerebrospinal fluid Cerebrospinal fluid (CSF) is a clear, colorless Extracellular fluid#Transcellular fluid, transcellular body fluid found within the meninges, meningeal tissue that surrounds the vertebrate brain and spinal cord, and in the ventricular system, ven ...

in the

. It appears in patients who have severe immune suppression; since the advent of

highly active antiretroviral therapy The management of HIV/AIDS normally includes the use of multiple antiretroviral drugs as a strategy to control HIV infection. There are several classes of antiretroviral agents that act on different stages of the HIV life-cycle. The use of mul ...

, its incidence has declined in these patients. The majority of PCNSL linked to HIV is Epstein-Barr virus positive. Furthermore, unlike HIV-negative PCNSL, patients typically present with multiple brain lesions and/or changes in mental status or focal neurologic symptoms. Changes in mental state,

intracranial pressure Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury ( mmHg) and at rest, is normally 7–15 mmHg for a supine adu ...

symptoms (

headache A headache, also known as cephalalgia, is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of Depression (mood), depression in those with severe ...

nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. It can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the throat. Over 30 d ...

vomiting Vomiting (also known as emesis, puking and throwing up) is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis, pre ...

papilledema Papilledema or papilloedema is optic disc swelling that is caused by increased intracranial pressure due to any cause. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare. In ...

), and local compression symptoms (

epilepsy Epilepsy is a group of Non-communicable disease, non-communicable Neurological disorder, neurological disorders characterized by a tendency for recurrent, unprovoked Seizure, seizures. A seizure is a sudden burst of abnormal electrical activit ...

memory loss Amnesia is a deficit in memory caused by brain damage or brain diseases,Gazzaniga, M., Ivry, R., & Mangun, G. (2009) Cognitive Neuroscience: The biology of the mind. New York: W.W. Norton & Company. but it can also be temporarily caused by t ...

, unstable gait,

visual impairment Visual or vision impairment (VI or VIP) is the partial or total inability of visual perception. In the absence of treatment such as corrective eyewear, assistive devices, and medical treatment, visual impairment may cause the individual difficul ...

, slurred speech) are the most common symptoms of PCNSL.

Primary effusion lymphoma Primary or primaries may refer to: Arts, entertainment, and media Music Groups and labels * Primary (band), from Australia * Primary (musician), hip hop musician and record producer from South Korea * Primary Music, Israeli record label Work ...

is a distinct subtype of aggressive

B-cell B cells, also known as B lymphocytes, are a type of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted or inserted into the plasm ...

NHL. It is brought on by

HHV8 Kaposi's sarcoma-associated herpesvirus (KSHV) is the ninth known human herpesvirus. It is also called Human herpesvirus 8, or HHV-8 in short. This virus causes Kaposi's sarcoma, a cancer commonly occurring in AIDS patients, as well as primary ef ...

, commonly referred to as KSHV, or Kaposi sarcoma-associated herpesvirus. It is uncommon, making up 0.3% of NHL in the general population and 4% of NHL linked to

. Between 60 and 90 percent of cases of

primary effusion lymphoma Primary or primaries may refer to: Arts, entertainment, and media Music Groups and labels * Primary (band), from Australia * Primary (musician), hip hop musician and record producer from South Korea * Primary Music, Israeli record label Work ...

have EBV co-infection, although its pathogenesis is unknown. There are malignant lymphomatous effusions in the

pericardium The pericardium (: pericardia), also called pericardial sac, is a double-walled sac containing the heart and the roots of the great vessels. It has two layers, an outer layer made of strong inelastic connective tissue (fibrous pericardium), ...

peritoneal cavity The peritoneal cavity is a potential space located between the two layers of the peritoneum—the parietal peritoneum, the serous membrane that lines the abdominal wall, and visceral peritoneum, which surrounds the internal organs. While situated ...

, and

pleural space The pleural cavity, or pleural space (or sometimes intrapleural space), is the potential space between the pulmonary pleurae, pleurae of the pleural sac that surrounds each lung. A small amount of serous fluid, serous pleural fluid is maintained ...

. Plasmablastic lymphoma (PBL) originates from terminally differentiated, activated

B-cells B cells, also known as B lymphocytes, are a type of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted or inserted into the plasm ...

at the post-germinal center that are changing from immunoblasts to

plasma cells Plasma cells, also called plasma B cells or effector B cells, are white blood cells that originate in the lymphoid organs as B cells and secrete large quantities of proteins called antibodies in response to being presented specific substances c ...

. About 2% of all AIDS-associated lymphomas are PBL-associated lymphomas.

infection is closely associated with this uncommon form of

, which primarily affects the oral cavity. With a widespread proliferation of massive neoplastic cells that resemble

immunoblasts but have the immunophenotype of

, it is incredibly aggressive.

Hodgkin lymphoma Hodgkin lymphoma (HL) is a type of lymphoma in which cancer originates from a specific type of white blood cell called lymphocytes, where multinucleated Reed–Sternberg cells (RS cells) are present in the lymph nodes. The condition was named a ...

(HL) is one of the most common cancers that do not indicate

, and since

was introduced, its incidence has increased. It is a germinal center-derived cell that produces Hodgkin Reed–Sternberg (HRS) cells. It is more common in

immunocompromised Immunodeficiency, also known as immunocompromise, is a state in which the immune system's ability to fight infectious diseases and cancer is compromised or entirely absent. Most cases are acquired ("secondary") due to extrinsic factors that affe ...

individuals, especially those with HIV. Compared to mild immune compromise, the incidence of HL is lower in states of extreme

. It's possible that there was insufficient immunological contact between non-neoplastic inflammatory cells and HRS cells.

Mechanism

-positive patients have a higher incidence of malignancies for a variety of reasons. These consist of

inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...

cytokine Cytokines () are a broad and loose category of small proteins (~5–25 kDa) important in cell signaling. Cytokines are produced by a broad range of cells, including immune cells like macrophages, B cell, B lymphocytes, T cell, T lymphocytes ...

dysregulation, and persistent antigenic stimulation. Moreover, oncogenic viruses are more likely to infect

patients. Thus, a variety of factors, such as a compromised

immune system The immune system is a network of biological systems that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to bacteria, as well as Tumor immunology, cancer cells, Parasitic worm, parasitic ...

, genetic changes, viral infection, and persistent

B cell B cells, also known as B lymphocytes, are a type of the lymphocyte subtype. They function in the humoral immunity component of the adaptive immune system. B cells produce antibody molecules which may be either secreted or inserted into the plasm ...

activation, contribute to the pathogenesis of

-associated lymphoma.

References

External links

Office of HIV and AIDS Malignancy in the National Cancer Institute

Information on AIDS-related cancers from the National Cancer Institute
{{Hematological malignancy histology HIV/AIDS Lymphoma

Signs and symptoms

Causes

Mechanism

References

Further reading

External links