Assessment 1 Flashcards
During the fetal period, definitive HPesis occurs in the?
LIVER AND SPLEEN
In adults HPesis confined to the?
Sternum
Vertebra
RIbs
List the hematopoiesis sites from development to adulthood hood
Derived from splanchnopleura (CD45+/CD34+) -> yolk sac (weeks 2-6 gestation; CD34+) -> liver and spleen (up until birth) -> long bones (tibia and femur) during childhood -> flat bones (ribs, sternum, vertebra) during adulthood
Most common precursor in hemopoietic tissue
Granulocyte/monocyte precursor
Describe how the major cell types are arranged relative to distance from sinusoids
principle - most migratory is furthest away from sinus
o Megakaryocytes – closest to sinuses
o Erythroid cells also close to the endothelium
o Leukocytes are farthest away from the sinus
-They have great migratory potential
What 2 progenitor cells does the common myeloid progenitor give rise to?
- Granulocyte/monocyte progenitor (CFU-GM)
2. Megakaryocyte/erythroid progenitor (MEP)
Granulocytosis
- stage with no granules
- last stage w/ nucleoli
- appearance of primary granules
- differentiating step
- last stage to undergo mitosis
- kidney bean shape
- myeloblast
- promyelocyte
- promyelocyte -> azurophilic granules (lysosomes)
- myelocyte -> 2ndary granules
- myelocyte -> last mitotic cell
- metamyelocyte
One bone marrow derived cell (CFU-GM) that undergoes mitosis in tissues?
monocyte: becomes a macrophage
JAK2 V617F mutation
- what does it indicate?
- what is it never seen with?
- If we see this we know there is a neoplasia
- But it’s not specific nor is it necessary to MPN
• Never seen with BCR/ABL
3 aspects of leukoerythroblastosis
-why/when does it occur with MPNs
- teardrop RBCs
- circulating nucleated red cells
- left-shifted granulocytes
Fibrosis of the bone marrow. Seen in late stages of the disease
ALL immunophenotyping
B-ALL
T-ALL
which one presents as a mediastinal mass?
Both are TdT positive
B-ALL -> CD19 and CD10 (CALLA)
T-ALL -> CD2/3
T-ALL -> mediastinal mass
APL -translocation? -cells filled with? -increased risk of? Treated with?
t(15;17)
Cells filled with auer rods (MPO+)
Inc risk of DIC -> auer rod/granule release
Txed with all-trans retinoic acid
Smudge cells are seen in which leukemia?
Immunophenotype for this cancer?
CLL/SLL
Immunophenotype
- CD19, CD20 (b-cell antigens)
- Abnormal CD5 (T cell antigen)
- CD23
Which leukemia is positive for TRAP?
presentation of this cancer?
Hairy cell leukemia
- ages 40 to 60
- Splenomegaly
- pancytopenia
- absence of lymphadenopathy
- B-cell leukemia
Target cells associated with?
HALT said the target cell
HbC disease
Asplenia
Liver disease
Thalassemia
Age distribution for HL vs NHL
prepubescent child?
HL
-bimodal: young adulthood and > 55 years
NHL
-peak for certain subtypes at 20-40
Prepubescent child more likely to have NHL
5 categories for lymphadenopathy
- Increase in # of lymphocytes and macrophages in response to antigens
– Infection or autoimmune - Infiltration by inflammatory cells/ infection of
LN “lymphadenitis” - Proliferation of malignant lymphocytes or macrophage
- Infiltration by metastatic malignant cells
- Infiltration by metabolite-laden macrophage (lipid storage disease)
Differentiate classical vs. non-classical HL by CD
-which HL in non-classical?
Classical -> CD30+, CD15+/-
Non-classical = nodular lymphocyte-predominant HL -> CD20+, CD45+
Describe the staging of Hodkin’s lymphoma
Stage 1 and 2 - good prognosis
I - single lymph node
II - 2+ regions on same side of diaphragm (mediastinal LN)
III - both sides of diaphragm or spleen involvement
IV - disseminated involvement of lymphatic tissue
Follicular lymphoma
- chromosome abnormality -> leads to?
- tx
t(14;18)
overexpression of bcl-2 = anti-apoptosis gene
Also CD10+
Chemotherapy
Rituximab -> anti-CD20 Ab
- helps NK cells bind and kill cancer cells
- used for maintenance tx
Burkitt lymphoma
- pathogenesis
- types
- histology
- tx and associated problems
African type
- EBV
- jaw lesion
Non-endemic
- t(8;14) -> B looks like 8
- activation of c-myc -> rapid proliferation of B-cells
- abdomen
- fastest proliferating tumor
HISTOLOGY
- starry night appearance
- night = b-lymphocytes
- stars = macrophages
- indicates rapid cell turnover
Tx -> tumor lysis syndrome
-give allopurinol and urate oxidase
Mantle cell lymphoma
- translocation
- CD
t(11;14)
-cyclin D1 -> increased cell cycle
poor prognosis
CD5+
H. pylori associated with which lymphoma?
indolent or aggressive?
MALT lymphoma -> indolent (low grade)
Most common form of aggressive lymphoma in adults?
DLBCL
-activated B-cell signature = worse prognosis
Marker for Burkitt Lymphoma
Ki-67 proliferation marker > 98%
Also CD10+
Nodular lymphocyte predominant HL
-histology/immunohistochem
CD20+ cells w/ rosettes of CD30+ T cells around the LP cell
“popcorn cells”
HL vs NHL based on neoplastic cells
HL -> few neoplastic cells
NHL -> most cells neoplastic
CRABBI for MM
C - hypercalcemia R - renal insufficiency A - anemia B - bone lesions B - bence jones proteinuria - Ig light chains in urine
Rasburicase for MM - MoA
urate oxidase -> converts uric acid into a soluble form
Paroxysmal nocturnal hemoglobinuira
Intravascular/intrinsic hemolytic normocytic anemia
Defect in GPI or DAF - protect RBC from complement
Neutrophils and platelets lysed too
triad - hemolytic anemia, pancytopenia, venous thrombosis
Labs: CD55/CD59 negative
tx w/ eculizumab
Hereditary spherocytosis
Defect in proteins interacting w/ RBC membrane skeleton and plasma membrane
-ankyrin, band 3, protein 4.2, spectrin
- inc MCHC -> concentrated Hb
- inc RCDW
Positive osmotic fragility test
Tx w/ splenectomy
Microangiopathic anemia
- path
- histo
path -> RBCs damaged when passing through obstructed or narrowed vessel lumina
-DIC, TTP-HUS, SLE
Histo -> shistocytes (helmet cells)
Macroangiopathic anemia
-due to
Prothetic heart valve and aortic stenosis -> mechanical destruction of RBCs
