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Hematopathology
Hematophathology
100
Pathology
Professional
06/06/2011

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Term
Leukemia/Lymphoma - which is characterized as a proliferation of neoplastic cells in the bone marrow?
Definition
Leukemia. Lymphoma is in the LNs.
Term
How is leukemia categorized?
Definition
Lymphoid or myeloid cells.
Term
What type of lymphoid cells are neoplastic in leukemia/lymphoma?
Definition
B/T/NK
Term
What type of myeloid cells are neoplastic in leukemia?
Definition
Only leukemia has myeloid cells - Granulocytes, monocytes, megakaryocytes, erythrocytes.
Term
Describe acute leukemia cells.
Definition
More common in younger pts, immature, classically have distinct lymphoid and myeloid cell origins, more progressive/aggressive.
Term
Describe chronic leukemia cells.
Definition
More common in older pts, more mature cells, more indolent, longer disease course.
Term
What is the site of involvement of AML?
Definition
Peripheral blood.
Term
What is AML?
Definition
Acute Myeloid Leukemia - monoclonal proliferation of immature usually blastic myeloid cells.
Term
Who gets AML?
Definition
Older people.
Term
Describe acute leukemia w/ t(15:17)(q22:q12) - what is it associated with?
Definition
Abnormal promyelocytes.
Term
What is APL?
Definition
Acute Promyelocytic Leukemia. Have bilobed dumbbell shaped nuclei.
Term
T/F APL cells are blasts.
Definition
No.
Term
What is the treatment for APL?
Definition
All-trans retinoic acid (ATRA) that lets the leukemic cells differentiate beyond the promyelocytic stage.
Term
What does APL cause?
Definition
Disseminated Intravascular Coagulation - DIC.
Term
What does treatment of APL w/ ATRA cause?
Definition
Thrombosis.
Term
What is AML, NOS?
Definition
AML, Not Otherwise Specified - so it includes all other myeloid cell types.
Term
What is MPN?
Definition
Myeloproliferative Neoplasms. These are abnormal clonal proliferations where excessive numbers of cells of 1 or more myeloid cell lines.
Term
What is the difference between MPN and AML?
Definition
Complete maturation of the cell lines.
Term
What causes the abnormal proliferation of MPN cells?
Definition
Intra-cellular signalling activated by Tyrosine kinases.
Term
Which tyrosine kinase is the prototype of MPNs?
Definition
BCR-ABL1 - it's activated and always turned on leading to abnormal proliferation.
Term
What chromosome is mutated in MPN?
Definition
Philadelphia chrosome.
Term
How is MPN treated?
Definition
Imanitab - specifically targes the BCR-ABL1 gene and its tyrosine kinase product.
Term
Why could someone with CML end up getting an acute leukemia w/ myeloid blasts or lymphoid blasts?
Definition
As CML progresses, there can be an accumulation of add'l cytogenetic abnormalities - cytogenetic evolution.
Term
What is found in cases of Polycythemia Vera (PV) Thrombocythemia (ET), and Primary Myelofibrosis?
Definition
JAK2 Used for diagnosis of MPN. This is a mutation. Without it, diagnosis was much more difficult.
Term
What is PV?
Definition
Chronic leukemia of RBCs.
Term
T/F Venous and arterial thrombosis can occur w/ PV.
Definition
True.
Term
T/F Hemorrhage and thrombosis both occur with PV.
Definition
False - ET.
Term
T/F Most pts w/ ET have JAK2 mutation.
Definition
false - just 50%.
Term
What is the replacement of bone marrow by fibrotic tissue?
Definition
Extramedullary hematopoeisis. Necessitates the production of RBCs elsewhere.
Term
Where does extramedullary hematopoesis typically occur?
Definition
Spleen/liver.
Term
What is effacement of lymph nodes?
Definition
Normal architecture is partially or completely effaced depending on where the lymphoma occurs. REPLACED AND OBLITERATED. Typical in Non-HL, also HL.
Term
What 2 patterns are seen in non-Hodgkin Lymphoma.
Definition
Diffuse and follicular.
Term
Which pattern of non-HL has B-cell involvement?
Definition
Follicular.
Term
What cells are involved w/ diffuse non-HL?
Definition
Diffuse - B/T/NK.
Term
Describe follicular non-HL.
Definition
Composed of cells w/ morphologies similar to benign germinal ctr-like structures. They form these center-like structures.
Term
Who typically gets ALL?
Definition
Kids.
Term
What is ALL?
Definition
Acute Lymphoblastic Leukemia.
Term
What is the predominant cell type in ALL?
Definition
B cells.
Term
What are the symptoms of ALL?
Definition
Fever (secondary to neutropenia), Bleeding (secondary to thrombocytopenia), Fatigue (secondary to anemia), Bone pain (secondary to infiltratin of the periosteum or compression fractures).
Term
What are the symptoms of LBL?
Definition
B-LBL - no bone marrow involvement - usually asymptomatic. T-LBL - Thymus involved. Skin, tonsils, LNs, liver, spleen, CNS also involved.
Term
What is LBL?
Definition
Lymphoblastic lymphoma.
Term
What is the primary site of involvement in ALL? Secondary?
Definition
Bone marrow, peripheral blood. Also CNS, Spleen, liver, testis.
Term
What is the primary site of involvement in LBL?
Definition
Lymph nodes. T-LBL - thymus, skin, tonsils, liver, spleen, CNS.
Term
With B-ALL/LBL - which has a better prognosis - Hyper or Hypo ploidy?
Definition
Hyper - typical in children, rare in adults. Hypo is more severe, not frequent in adults or kids.
Term
What chromosome is the precursor for ALL/LBL neoplasms?
Definition
Philadelphia - causes BCR-ABL2 mutations. Same as in CML except ABL2 instead of 1.
Term
Is the transolaction of the Philadelphia chromosome more frequent in kids or adults?
Definition
Adults - not frequent at all in kids. Unfavorable prognosis.
Term
What is the sanctuary site for ALL/LBL? Why?
Definition
Testis and CNS - impervious to chemotherapeutic agents.
Term
What is the most common leukemia in the Western world?
Definition
CLL.
Term
What symptoms do most people with CLL have?
Definition
Asymptomatic typically, but if symptoms present - lymphadenopathy or hepatosplenomegaly. Also anemia, neutropenia, or thrombocytopenia.
Term
Who gets CLL?
Definition
Older people.
Term
What is the predominant cell in CLL/SLL?
Definition
Small, round to slightly irregular B-cell. There are also larger B-cells w/ nucleoli called prolymphocytes and para-immunoblasts.
Term
How are CLL/SLL diagnosed?
Definition
Flow cytometry.
Term
Who gets Follicular Lymphoma?
Definition
Older people.
Term
What is the cell of origin of FL?
Definition
Germinal center cells.
Term
What is the disease process w/ FL?
Definition
Germinal center cells contain heterogenous collection of many diff. cells, also macs, containing apoptotic debris, dendritic cells, T-cells.
Term
What cells are contained in FL neoplastic germinal centers?
Definition
More monotonous pop - centrocytes and centroblasts - but usually cytes.
Term
T/F the neoplastic cells in FL germinal ctrs are packed loosely.
Definition
False - packed tightly - lack the cuff of small mature lymphocytes that surround normal germinal ctrs. Thus when lymph node architecture becomes effaces by neoplastic germinal ctrs - it's called follicular.
Term
T/F The higher the grade of FL - the more centroblasts that are present.
Definition
True - then it behaves more aggressively w/ more blasts.
Term
T/F Diffuse large B-cell lymphoma (DLBCL) is most common in kids.
Definition
False - elderly.
Term
What is the cell of origin in DLBCL?
Definition
Many cells that arise from diff. points of antigen stimulated B-cell development.
Term
What is the morphology of DLBCL?
Definition
Large - bigger than a mac nucleus - size of 2 mature small lymphocytes.
Term
What B-Cell antigens are typical in DLBCL?
Definition
CD19, CD20, and antigens that reflect the pt of antigenic stimulated dev. where they arose.
Term
T/F Burkitt Lymphoma is benigh.
Definition
False very aggressive. 2-3 days can cause death if near trachea.
Term
T/F Burkitt lymphoma is a neoplasm of B-cells.
Definition
True.
Term
What are the 3 types of BL?
Definition
Burkitt Lymphoma - Endemic BL, Sporadic BL, Immunodeficient BL.
Term
What are areas of involvement w/ Endemic BL?
Definition
Jaws - other facial bones.
Term
Who is affected with Endemic BL?
Definition
Children - mostly in Africa.
Term
What virus is seen with BL?
Definition
EBV.
Term
What is the site of infection w/ Sporadic BL?
Definition
Ileo-cecal area - leading to presenatation w/ an abdominal mass.
Term
What causes Immunodeficient BL?
Definition
HIV/AIDS.
Term
What is the cell of origin of BL?
Definition
Small non-cleaved germinal center cell.
Term
What is the morphology of a BL cell?
Definition
Medium sized cell w/ basophilic cytoplasm that contains prominent vacuoles. Nucleus is round and contains finely clumped chromatin and multiple medium sized nucleoli.
Term
What are the immunophenotypic findings w/ BL?
Definition
B-cell antigens and antigens that reflect its germinal ctr origins.
Term
What is M-protein?
Definition
Monoclonal plasma cells that all secrete a single Ig or portions that are the same size.
Term
What is the order of frequency of diff. types of paraprotein in myeloma?
Definition
IgG > IgA > Bence Jones protein > IgD > IgE > IgM.
Term
What is an M-spike?
Definition
Igs found in the gamma region. Normal people have a smear, people w/ monoclonal populations have a spike that is a single dark band.
Term
What is CRAB associated with?
Definition
PCM
Term
What is CRAB?
Definition
End organ dmg from PCM - hyperCalcemia, Renal failure, Anemia Bony lesions.
Term
T/F Patients w/ Smoldering Myloma have end organ dmg.
Definition
False - only those w/ PCM.
Term
What are the symptoms of SM?
Definition
Smoldering Myeloma - asymptomatic. Can progress to PCM.
Term
What is the site of proliferation of PCM and SM?
Definition
Bone marrow - both involved and uninvolved areas.
Term
What do cells of PCM look like?
Definition
Abnormal - atypical - immature.
Term
What are the radiographic findings with PCM?
Definition
Osteoclasts are activated - so lytic bony lesions are a feature of PCM.
Term
What is the immunophenotypic finding w/ a proliferation of neoplastic plasma cells (PCM)?
Definition
Only kappa/lambda light chains in the cytoplasm due to monoclonal Igs.
Term
What's the difference btw PCM and SM?
Definition
Presence of end organ dmg. With SM you nee dto see monoclonal plasma cell proliferaiton for SM to diagnose (w/ no end organ dmg).
Term
What is the cell of origin of a NLPHL?
Definition
Germal ctr, centroblastic B-cell.
Term
What is the morphology of NLPHL cells?
Definition
Large folded multilobulated nuclei w/ a small nucleolus.
Term
What disease has "popcorn cells"?
Definition
NLPHL - Large folded, multi-lobulated nuclei w/ a small nucleolus.
Term
What cells are predominant in an NLPHL LN?
Definition
Small histocytes, dendritic cells, popcorn cells.
Term
What antigen do LP cells exhibit?
Definition
CD45. Called lukocyte common antigen b/c it's express by almost all myeloid and lymphoid cells.
Term
T/F LP (Popcorn cells) cells express CD20, CD15, CD45, and CD30.
Definition
False - Only 20 and 45, not 15 or 30. These are expressed by CHL cells.
Term
What accounts for the vast majority of HL?
Definition
CHL. Hence classical.
Term
What is the morphology of CHL cells?
Definition
2 kinds - Reed Steinberg and Hodgkin cells. Collectively called HRS cells.
Term
What is the cell of origin of CHL cells?
Definition
B- cells in germinal centers.
Term
Which cells are diagnostic in CHL?
Definition
Reed Steinberg.
Term
What is the shape of Reed-Steinberg cells?
Definition
Large w/ large bilobed nucleus, eosinophilic. Central nucleolus w/ a clear area surrounding the nucleolus.
Term
What cell has pink cytoplasm?
Definition
Reed-Steinberg cells.
Term
What is the diff. between a Reed-Steinberg cell and a Hodgkin Lymphoma cell?
Definition
The same, except a HL cell has a non-lobated nucleus.
Term
T/F CHL Hodgkin cells are in the majority.
Definition
False - in minority to inflammatory cells that surround it. Include eosinophils, lymphocytes, plasma cells and histiocytes.
Term
Which, CHL or NLPHL has a full-fledged B-cell immunophynotype?
Definition
NLPHL.
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