Hodgkin Lymphoma Flashcards
(39 cards)
What are lymphomas? What are the two types?
Lympohas: group of malignant disorders arising primarily in the lymph nodes
Lymphoma types: Hodgkin and Non-hodgkin. Other rarer types.
Hodgkin lyphoma
characterised by lymphadenopathy and presence of Reed Srernberg (RS) cells
Hodgkins Lymphoma disease course
- Maligant cells accumulat ein signle lymph node, may eventually spread to other nodes
- May also enter blood stream and infiltrate organs
- outcome of HL patients improved over the last few decades: late effects of disease are more apparent now
- Consdiered curable in majority of cases
HL incidence
-Uncommon
- Present at any age
- Annual incidence of HL 3/100,000
- incidence peak in young adults 20-34, further peak observed >70 years of age
- Incidence is currently stable
HL Risk factors
- No one single clear-cut causative agent but the following is implicated:
The Epstein-Barr virus has been identified as being a possible cause of HL.
Previous NHL
Hepatitis C
Exposure to pesticides
Poor immunity (HIV, post-transplant, rare immunological disorders)
Higher socioeconomic status and lack of early exposure to infections
Family history (could be due to inheritance or shared lifestyle factors)
May be lower risk in alcohol drinkers but not in drinkers who also smoke
Studies ahoq increased risk of Hodgkin lymphoma for people who are obese
Many people do not have any identifiable risk factors
HL Pathogenesis
- Not fully understood
- RS Cells: central to diagnosis of four classic types (large multi-nucleate cells)
- Mononuclear Hodgkin cells also part of the malignant clone
- Possess mechanisms preventing apoptosis
-Malignant cells and inflitration of inflammatory cells present
-RS cell is B-cell lineage showing clonal rearrangement of immunoglobulin genes.
HL symptoms
Enlarged painless lymphadenopathy
The nodes often fluctuate in size
Alcohol ingestion may precipitate pain
Itching
Cough or breathlessness
Hepatic and splenic enlargement may occur
Systemic symptoms including fever, weight loss, fatigue and night sweats
Extra nodal disease: lung, skin, CNS and bone marrow involvement may occur
Infection may occur due to defective humoral/cell mediated immunity
HL Clinical Features
G
what are HL B symptoms? Examples
B symptoms: (named after the B lymphocytes, which are involved in the immune response) are Systemic symptoms of drenching night sweats, unexplained fever >38°C, and weight loss of >10% over 6 months are termed B symptoms and are identified in approximately 25% of patients.
HL Clincal Features
- HL can arise in any part of the lymphatic system, more common in: Cervical nodes: 60-70% of patients, Axillary nodes: 10-15% of patients, Inguinal nodes: 6-12% of patients
-Mediastinal disease: identified in 80% of patients and is more common in nodular sclerosing HL - peripheral or sub-diaphragmatic lymphadenopathy :more common in mixed-cellularity classical HL.
- Bone marrow involvement: detected in only 5–8% of patients with conventional staging-up to 18% with positron emission tomography (PET)/computerized tomography (CT) staging
- many patients have painless swelling in neck/ armpit / groin
- small proprotion of patients have signs of extra nodal disease at presentation, typically in liver and bone marrow
- Bone marrow infiltration associated with specific symptoms
Classification
-Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL)
-Classical Hodgkin Lymphoma (cHL):
Nodular sclerosis classical Hodgkin lymphoma
Mixed- cellularity classical Hodgkin lymphoma
Lymphocyte rich classical Hodgkin lymphoma
Lymphocyte depleted classical Hodgkin lymphoma
cHL and NLPHL characteristics
- both types characterised by presence of small number of neoplastic cells in inflammatory background of reactive T cells and eosinophils. Varying amounds of fibrosis present.
Diagnosis of HL (NLPHL and CHL typical characteristics for diagnosis)
- histological examination of a lymph node biopsy (microscopy with immunohistochemical staining) - Antibodies include: CD15, CD30, CD20 and CD45.
NLPHL:
RS absent but distinct histological appearance.
Cells having a lymphocytic and histiocytic or ‘popcorn’ appearance – called lymphocyte predominant cell (LP-cell)
The immunophenoytype is more typical for a B cell, being positive for CD20 and immunoglobulin expression.
Cells usually CD45 positive and negative for CD15 and CD30.
CHL:
Divided into 4 subtypes, based on RS morphology and composition of reactive cell infiltrate.
Nodular sclerosing is the most frequent.
CD 20 negative.
CD30 + / CD15 +/-
RS cell formation/ action
- derived from germinal centre B cells with mutations of the IgH-variable region segment.
- usually aneuploid with no consistent cytogenetic abnormality.
- secrete cytokines to recruit reactive cells that include IL-5 and transforming growth factor-beta (TGF-beta).
- Clonal Ig gene rearrangements found in majorit of isolated RS cells
RS cells - Immunohistochemistry stains results
-positive for CD30 and CD15 but typically negative for CD20 and CD45, which are positive only in neoplastic NLP-HL cells.
- also usually positive for PAX5, CD25, HLA-DR, ICAM-1, Fascin, CD95 (apo-1/fas), TRAF1, CD40, and CD86.
RS cell variant examples
-Hodgkin cell, mummified cells, and lacunar cells.
- Hodgkin cells are mononuclear RS-cell variants.
NLPHL: LP cells immunohistochemistry stain results
NLPHL: LP cells : usually positive for C020, CD45, EMA, CD79a, CD75, BCL6, BOB.1, OCT2, and J chain.
Lab features of HL
Peripheral blood manifestations (non-specific)
Anaemia (normocytic, normochromic)
Eosinophilia frequent
Neutrophilia (one-third of patients)
Low lymphocyte count in advanced disease
Platelets (normal or increased in early-stage disease and low in late-stage disease)
Bone marrow involvement may be detected
Raised ESR and CRP: useful prognostic markers and for monitoring response to treatment
LDH is sometimes raised initially
Abnormal LFT’s and U&E’s
Raised urate levels
Staging of Hodgkin Lymphoma (stage 1)
- node involvement in one lymph node area.
Staging of Hodgkin Lymphoma (stage 2)
disease involving two or more lymph nodal areas confined to one side of the diaphragm.
staging of Hodgkin Lymphoma (stage 3)
disease involving lymph nodes above and below the diaphragm. Splenic disease is included in stage Ill but this has special significance
Staging of Hodgkin Lymphoma ( A, B. further staging classifications)
- A is absence and B is presence of one or more of the following: Unexplained fever above 38 degrees celcius, ight sweats, loss of more than 10% body weight within 6 months
- E: Localised extra nodal extension from mass of nodes, doesnt advance stage
IE- medistinal disease with contiguous spread to the lung/ spinal theca
IIIs : for patients with lymphy node and splenic involvement - spleen involvement usually seen before widespead haematogenous spread of HL
Bulky disease: wideneing of mediastinum by more than 1/3 or presence of nodal mass > 10 cm in diameter. This is revelant to therapy at any stage
staging of Hodgkin Lymphoma (stage 4)
involvement outside the lymph node areas and refers to diffuse or disseminated disease in the bone marrow, liver and other extra nodal sites