Lymphomas Flashcards
What are lymphomas
Neoplasms of lymphoid origin, typically causing lymphadenopathy
According to WHO in 2001, lymphomas are classified into
Hodgkin’s and Non hodgkin’s
Non hodgkins lymphoma are further divided into
B-cell neoplasms :precursor and mature
T cell and Nk neoplasms: p and m
Risk factors for NHL
FI³C
Family history of lymphoma
Ionising radiation
Infectious agents
Immunosuppression
Connective tissue disease
Clinical manifestations of lymphoma
Be very SLICK when talking about lymphoma: V-SLC
VARIABLE
Could be symptomatic or asymptomatic
Time course- could evolve over weeks, months,even years.
SYSTEMIC MANIFESTATION
Constitutional symptoms like fever, night sweats,weight loss
LOCALISED MANIFESTATION
lymphadenopathy,splenomegaly most common
Any tissue can be infiltrated
Other COMPLICATIONS
Immune hemolysis
Bm failure
Cns infiltration pleural/pericardial effusions
For lymphomas, diagnosis MUST be biopsy proven before initiating therapy and must be Confirmed by Immunopenotyping. T/F
T
Gold standard for lymphoma diagnosis
Excisional/incisional biopsy
What are the trephine methods that can be used for lymphoma diagnosis
Core needle vs open vs fine needle aspiration biopsy
Necessary because enough tissue is needed to assess cells and architecture
Staging of hodgkins lymphoma
?
The three common lymphomas include, which is the commonest
Follicular lymphoma
Diffuse large b cell lymphoma -commonest
Hodgkin’s lymphoma
What are the indolent lymphomas
Slowly follow the margin
Small lymphocytic lymphoma
Lymphoplasmacytic and Waldenstrom macroglobulinemia
Follicular lymphoma
Marginal zone Bcell malt and nodal lymphoma
Most common indolent lynphoma
Follicular lymphomas
Hodgkin’s lymphoma was discovered by ?
Thomas Hodgkins
Cell of origin of hodgkin’s lymphoma
Germinal center B cells
Remember the REAL classification of hodgkins lymphoma
Classical and non classical
In hodgkin’s lymphoma, most cells are neoplastic cells rather than polyclonal reactive lymphoid cells . T/f
F…most are polyclonal reactive lymphoid cells
Epidemiology of hodgkins lymphoma
Less frequent than NHL
Male predilection
Peak incidence at 3rd decade of life
Associated or etiology factors of hodgkin’s lymphoma
Shope is the cause of hodgkin’s lymphoma
S- small family size
H-higher socioeconomic status
O-others like hiv, herbicides,occupation
P-possible genetic predisposition
E-EBV infection
The bimodal age distribution of hodgkins lymphoma is between
20-30yrs for young adults
>50 yrs for elderly
However may occur at any age
Describe the lymphadenopathy in hodgkin’s lymphoma
CAPENERs SIGN
C-Cervical region
A-Asymmetrical, discreete
P-Painless, non tender
E-Elastic character on palpation
N-Non adherent to skin
E-fluctuant in size
R-Rare extranodal involvement
S- Spleen and liver enlargement
Investigations for hodgkins lymphoma
Fbc- anemia (normochromic, normocytic) , neutrophils, eosinophilia, lymphopenia
Raised Esr
Lft and renal fxn test before therapy
BMA
Increased urate, calcium
On doing chest x-ray, a mediastinal mass was found. This is typically seen in what lymphoma
Hodgkins
Clinical manifestations of hodgkins lymphoma
Lymphadenopathy
Contiguous spread
Rare extra nodal involvement.
B symptoms
3Ps: pallor
Pruritus
Pain at the site of disease after drinking alcohol
What are the B symptoms
Drenching night sweats
Sustained fever>38 degrees
Weight loss>10% of body weight in 6 months
The fever in hodgkins lymphoma is sometimes referred to as
Cyclical. Pel Ebstein fever
Pel Ebstein fever is a feature of
Hodgkin’s lymphoma
Most suitable treatment regimen for hodgkin’s lymphoma
A-adreomycin
B-bleomycin
V-Vinblastin
D-dacarbazine
Others include
M-nitogen mustard
O-oncoven
P-prednisone
P-Procarbazine
Copp
The ABVD regimen poses a higher risk of developing infertility than MOPP and thus counselling should be included in therapy. T/f
F…Mopp rather has a higher risk
Complications of treatment
Infertility
Premature menopause
Secondary Malignancy like skin aml breast etc
Cardiac disease
Hodgkins lymphoma is more common than non hodgkin’s. T/f
F
Epidemiology of NHL
Median age-65-70yrs
M>F
B-cell 70%..T cell 30%
When On Snapchat, Remember the clinical features of NHL
Widely disseminated at presentation
Hepatosplenomegaly.
Extranodal involvement
Nodal involvement
On …most common
Snap..Systemic symptoms
Chat ….compression syndrome
Extranodal involvement of Nhl
Intestinal lymphoma-abd pain, dysphagia, anemia
Cns- headache, cranial nerve palsy, sc compression
Lung, skin, testes,thyroid
Bone marrow, low grade
Systemic symptoms of Nhl
Sweating
Weight loss
Pruritus
Metabolic complications like hyperyricemia, calcemia,renal failure
Compression syndrome In Nhl
Gut obstruction
Ascites
Svc obstruction
Spinal cord compression
Investigations for Nhl
Similar to HD
Bma +aspiration
Immunophenotyping
B cell antigens(CD19, 20,22)
T cell antigens(CD 2, 3,5,7)
Hiv
Systems of classification of NHL
REAL
Clinical/working formulation;
Low grade? Remember
Intermediate
High grade?
Etiology of Nhl
Cannot be attributed to a single cause
Chromosomal translocation t(14,18)
Infections like EBV,HIV,HTLV,HHV8
Bacteria like H.pylori in gastric lymphoma
Immunodeficiency
Management of Nhl
Asymptomatic, no treatment
Radiotherapy for localised disease(stage 1)
Chemotherapy: Chlorambucil is the mainstay, however repeated relapses median survival 6-10yrs
Fludarabine, 2 cda newer
Rituximab
Sct/Bmt
Treatment regimen for high/intermediate grade Nhl
R-CHOP
R Rituximab
C cyclophosphamide
H doxorubicin hydrochloride
O-oncovin/vincristine
P prednisone
Other chemotherapy combos for nhl
Rice
Coap.
Epoch
Distribution of nodes for nhl is centripetal whereas HD is centrifugal t/f
F…HD is centripetal
Differences between hl and nhl
Look at picture
Nhl illustrates the concept of lymphoma/leukemia .t/f
True
Two important things to know about Burkitts
The neoplastic cells are B lymphoblasts with translocations of c-myc oncogene in chromosome 8q
Characteristic starry sky appearance
The two types of burkitts are? What are their differences
Endemic and non endemic(sporadic)
Differences..5
The doubling time of burkitts lymphoma is about
26hrs
Clinical features of burkitts lymphoma
Usually involves greater than 1 site at presentation
Commonly mandible,maxilla, abdomen and pelvis, gonads
Destruction of jaw bones- loosening of teeth
Dental anarchy and loss of dental lamina dura
The most frequent Cytogenetics translocation in burkitts
t(8,14)
Others include t(2,8)..t(8,22)
Pathogenesis of Burkitts lymphoma
Cmyc oncogene is translocated on a b lymphocyte as a chance event, probably a mutation
Ebv infects ad immortalises the b cells
Malaria stimulates the immortalised b cells o proliferate and produce antibodies. Leading to uncontrolled proliferation of b cell clone -burkitts lymphoma
Treatment of burkitts lymphoma
Prevent and treat tumor lysis effects
Endemic burkitts- Coap
Cyclophosphamide
Oncovin
Cytosine arabinosie
Prednisone
With intrathecal methotrexate or cytosar