Lymphoma Flashcards
(8:14) translocation
Burkitt lymphoma
endemic in areas of Africa
Burkitt lymphoma
arises from the naïve B‐cells in the mantle zone area of the lymphoid follicle
Mantle cell lymphoma
Trisomy chromosome 2, 7, 10
good prognosis for ALL
It characteristically arises in epithelial tissues such as stomach, salivary glands, GI tract lungs and the orbit.
Extra nodal Marginal Zone B‐cell lymphoma
In some patients transformation to a more aggressive lymphoma occurs (e.g. Diffuse Large B‐cell lymphoma).
Follicular lymphoma
MYC translocations
diffuse large B‐cell lymphoma; with this mutation, these are particularly aggressive tumors
80% cure rate with ALK inhibitors
Anaplastic Large‐Cell Lymphoma(ALK+)
WBC count in ALL
increased
cluster around venules
Anaplastic Large‐Cell Lymphoma
What are the two subgroups of CLL? Which has a worse prognosis?
One group arises from post germinal center cell origin (Ig geneshypermutated), and one group arises from naive B-cells. The latter group, arising from naive B cells has a worse prognosis
tumor of CD4 cells
Cutaneous T‐cell lymphoma aka mycosis fungiodes
CD103 and annexin
markers of hairy cell leukemia.
This tumor is characterized by the (11:14) translocation .
Mantle cell lymphoma
powerful transcription regulator and it helps promote aerobic glycolysis and rapid cell growth
MYC; refers to Burkitt lymphoma and diffuse large b-cell lymphoma
Risk of transformtion to DLBCL
extranodal marginal zone lymphoma; follicular lymphoma, CLL/SLL,
Somatic hypermutation: memory B‐cell origin
extranodal marginal zone lymphoma
presents as enlarging mediastinal mass in younger women.
Mediastinal large B‐cell lymphoma; type of diffuse large B-cell lymphoma
fastest growing human tumor
Burkitt lymphoma
up regulation of the anti‐apoptotic protein BCL‐2
CLL/SLL
associated with HIV
Burkitt lymphoma, DLBCL - primary effusion lymphoma
occurs in patients with T‐cell immunodeficiency such as HIV and allogenic bone marrow transplant cases
immunodeficiency associated diffuse large B‐cell lymphoma
tumor cells infected with KSHV/HHV‐8
primary effusion lymphoma; type of diffuse large B‐cell lymphoma
present as a new enlarging abdominal mass which runs a much more aggressive course
diffuse large B‐cell lymphoma –> Richter’s syndrome transformed from CLL/SLL
Aggressive tumors
• Generalized lymphadenopathy
• Pruritis
• Eosinophilia
• Weight loss
Peripheral T cell Lymphoma
The tumor cells express pan B cell antigens CD19, CD20 and co‐express CD5
Mantle cell lymphoma; CLL/SLL
The tumor runs a rapid and fatal course without any treatment.
Diffuse large B‐cell lymphoma
Normal reactive processes are ___clonal
poly
Most of these cases show EBV coinfection.
diffuse large B‐cell lymphoma
This is a fusion of the (1) gene on chromosome 18 with the (2) locus on chromosome 14.
- BCL 2. IgH follicular lymphoma; some cases of Diffuse Large B‐cell lymphoma
translocations of the MYC gene on chromosome 8 mostly with the IgH gene on chromosome 14
Burkitt lymphoma
The tumor cells have a characteristic “cerebriform “appearance.
Cutaneous T‐cell lymphoma aka mycosis fungiodes
Acute lymphoblastic leukemia represents 80% or more of childhood leukemia and most are of (1)‐cell origin
- B
Bone marrow is often not involved in contrast to some of the other lymphomas we have discussed so far.
Diffuse large B‐cell lymphoma
malignant clone is ___clonal
mono
collections of malignant CD4 positive T‐Cells
Pautrier’s microabscesses; seen in Cutaneous T‐cell lymphoma aka mycosis fungiodes
associated with EBV infection of the neoplastic elements due lack of T cell immunity(can regress when restored)
DLBCL
relatively uncommon B‐cell neoplasm characterized by cells with characteristic hair like cytoplasmic extensions
Hairy cell leukemia
Most cases will show peripheral blood involvement as the prominent process and thus the term (1) is used. When lymphadenopathy is the prominent process the term (2) is used.
- chronic lymphocytic leukemia 2. small lymphocytic lymphoma
TdT
expressed in both preB and preT cells; differentiates ALL from AML
autoimmune hemolytic anemia or autoimmune thrombocytopenia
CLL/SLL patients may develop autoantibodies
– Large cells 5x size of normal lymphocyte
– Prominent nucleoli
– Numerous mitosis
Diffuse Large B‐cell lymphoma
Other s/s of CLL
hepatosplenomegaly
What are the clinical features of ALL?
due to replacement of normal bone marrow cells with the leukemic cells: anemia, infections, petechiae, bruising, bleeding; bone pain, hepatosplenomegaly, lymphadenopathy, testicular enlargement, CNS involvement in children
T-ALL: mediastinal mass
Most patients with CLL will show an elevated (1) count consisting mostly of (2), with reduced numbers of?
- white blood cell 2. small well differentiated lymphocytes. 3. platelets, RBCs, neutrophils
Small well diff. lymphocytes and smudge cells
CLL/SLL
Some cases of CLL/SLL will over time transform into (1) and present as a new enlarging abdominal mass which runs a much more aggressive course.
- diffuse large B‐cell lymphoma
B‐cell neoplasms expressing T cell marker CD5
CLL/SLL and mantle cell lymphoma
Apoptotic cells “Starry Sky” Appearance
Burkitt lymphoma
ALL
painless generalized lymphadenopathy in patients over 50 years old.
Follicular lymphoma
“starry sky” appearance
Burkitt lymphoma
Bone marrow involvement either focal or diffuse is commonly seen.
Follicular lymphoma
Gain of function mutation NOTCH1
T cell ALL and CLL/SLL
CD25+ (IL‐2 receptor)
Adult T cell lymphoma/leukemia
erythroderma which may progress to plaque formation and finally to tumor formation in the skin
Cutaneous T‐cell lymphoma aka mycosis fungiodes
Diffuse infiltrate large lymphocytes; Numerous apoptotic bodies; Starry sky appearance
Burkitt lymphoma
arises from germinal center B -cells
follicular lymphoma
CyclinD1 thus is upregulated, promoting cell cycle progression from G1 to S‐phase.
Mantle cell lymphoma
How do you distinguish CLL from mantle cell lymphoma?
11:14 translocation and cyclin D1 overexpression in mantle cell lymphoma
Primary effusion lymphoma is another subtype occurring also in HIV patients.
diffuse large B‐cell lymphoma
It is a disease of older men who present with (1) from tumor involvement of these sites.
- pancytopenia and massive splenomegaly; refers to hairy cell leukemia
Mantle cell lymphoma presents with?
weakness, fatigue and generalized painless lymphadenopathy. Widespread involvement of bone marrow, liver, spleen and the gastrointestinal tract is common.
Deletions 11q and 17q are associated with worse prognosis
CLL/SLL
Widespread involvement of bone marrow, liver, spleen and the gastrointestinal tract is common.
Mantle cell lymphoma
In follicular lymphoma there are no (1) found within the follicles which themselves are composed of nodular aggregates of neoplastic lymphocytes.
- apoptotic bodies
BCR-ABL fusion gene from a (9;22) translocation is associated with a good prognosis
ALL
Some tumors show trisomy 12 or deletions of 11, 13, or 17.
CLL/SLL
activating BRAF mutations
Hairy cell leukemia
The average age at presentation is 60 years but notably the disease accounts for about 15 % of childhood lymphomas.
diffuse large B‐cell lymphoma
Refractory cases respond well to BRAF inhibitors.
Hairy cell leukemia
Lack of apoptotic bodies in the node
Follicular lymphoma
If localized, can be treated with excision
Extra nodal Marginal Zone B‐cell lymphoma
In the young adult patients (15‐20 yrs. old) the tumors are more frequently of the (1) cell type.
- pre‐ T refers to ALL
Which is more prominent in CLL/SLL? Tumor cell growth or survival?
Prolonged cell survival
Rituximab (anti‐CD20) is very effective in controlling the disease
Follicular lymphoma
Platelet count in ALL
decreased
The pan B‐cell markers are expressed on the tumor cells and the MYC translocation is identified in all cases.
Burkitt lymphoma
(14:18) chromosomal translocation.
follicular lymphoma and some cases of Diffuse Large B‐cell lymphoma
Tumor cells will show the pan B‐cell makers CD19, CD20 and also express CD11c and CD103 which usually are absent from other B‐cell neoplasms.
Hairy cell leukemia
The disease spreads from the initial skin involvement to lymph node and visceral involvement.
Cutaneous T‐cell lymphoma aka mycosis fungiodes
The more common lymphomas are by far of (1)‐cell origin and occur due to errors during the normal process of (2) in (1)‐cell differentiation and development
- B 2. somatic hyper mutation and immunoglobulin class switching
Anti-CD20 therapy is generally used (i.e. rituximab)
CLL/SLL
Those of pre‐ B cell origin originate in the (1)
- bone marrow; refers to Acute Lymphoblastic Leukemia/Lymphoblastic Lymphoma:
presents as a rapidly enlarging mass at nodal or commonly extra nodal sites almost anywhere in the body (skin, bone, brain and GI tract, tonsils, liver and spleen)
Diffuse large B‐cell lymphoma
mutations of BCL6, involved in normal germinal B‐cell maturation and growth arrest and apoptosis.
diffuse large B‐cell lymphoma
The tumor cells express the pan B cell markers CD19, CD20, BCL6 AND BCL10.
follicular lymphoma
Which are larger? Myeloid blast cells or lymphoblast cells?
Myeloid
many patients suffer infections from hypogammaglobinemia
CLL/SLL
Patients may remain in this erythroderma phase for long periods without spreading to viscera.
Sezary syndrome
Those associated with Pylori can often be effectively treated using antibiotics.
Extra nodal Marginal Zone B‐cell lymphoma
consists of medium to large size tumor cells with numerous mitotic figures and many apoptotic bodies from the rapid cell growth and turnover.
Burkitt lymphoma
ALK gene rearrangements‐tyrosine kinases activity
Anaplastic Large‐Cell Lymphoma(ALK+)
Retrovirus caused tumor of CD4+ cells; HTLV-1
Adult T cell Leukemia/Lymphoma
B cell markers such as (1) and T cell markers such as (2) allow further separation of the lymphoblastic leukemia into B and T cell types.
- CD19 2. CD3
RBCs in ALL
decreased: anemia
Endemic types all latently infected with?
EBV; Burkitt lymphoma
Those of pre‐T cell origin occur as (1) masses as they arise in the T‐cell areas of the (2) but eventually evolve to a (3) phase involving the peripheral blood.
- mediastinal 2. thymus 3. leukemic refers to pre-T cell ALL
Hematopoietic stem cell transplantation is used in selected patients with some success.
Mantle cell lymphoma
over expression of BCL2 protein and therefore increased cell survival.
follicular lymphoma
The cells are large and express the pan B‐cell markers CD19, CD20 and sometimes BCL6 as noted above.
diffuse large B‐cell lymphoma
Constitutional symptoms of fatigue, weakness anorexia and weight loss may be present along with recurrent infections.
CLL/SLL
patient with erythroderma and circulating malignant CD4 cells in the peripheral blood.
Sezary syndrome
lost function of PAX5
B cell ALL
very aggressive tumors occurring frequently in children and younger adults.
Acute Lymphoblastic Leukemia/Lymphoblastic Lymphoma:
This is a fusion of the cyclinD1 gene and the IgH locus.
(11:14) translocation Mantle cell lymphoma
Usually begins at extranodal sites such as maxillary/mandibular masses or abdominal tumors of bowel, ovary and
retro peritoneum
burkitt lymphoma
In SLL the prominent feature is (1) and biopsy of the involved lymph nodes will reveal complete replacement of the lymph nodes with these small well differentiated lymphoid cells
- generalized lymphadenopathy
Skin lesions; Hepatosplenomegaly; Lymphadenopathy; Hypercalcemia; Circulating tumor cells
Adult T cell Leukemia/Lymphoma
found frequently in patients with autoimmune disorders (1) and in patients with chronic infections (typically H. Pylori gastritis).
- Sjogren syndrome or Hashimoto thyroiditis; refers to Extra nodal Marginal Zone B‐cell lymphoma
multifocal submucosa nodules
(lymphomatoid polyposis) in GI tract
Mantle cell lymphoma
autoantibodies to RBCs and platelets leading to?
autoimmune hemolytic anemia and autoimmune thrombocytopenia; refers to CLL/SLL
commonly at extra nodal sites such as the maxillofacial areas (endemic cases) or gastrointestinal tract (sporadic cases)
Burkitt lymphoma