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Pathoma
WBC related disorders
99
Pathology
Graduate
02/24/2014

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Term
low WBC
Definition
< 5K; leukopenia
Term
high WBC
Definition
>10K; leukocytosis
Term
causes of neutropenia
Definition
drug toxicity (alkylating agents); severe infection (gram neg sepsis)
Term
what drugs can boost granulocyte production?
Definition
GM-CSF or G-CSF
Term
causes of lymphopenia
Definition
immunodeficiency (DiGeorge, HIV), high cortisol state (induced apoptosis), autoimmune destruction (SLE), whole body irradiation (highly sensitive)
Term
causes of increased neutrophils
Definition
bacterial infection/necrosis = release of marginated pool and bone marrow Nt; high cortisol state = impairs leukocyte adhesion, leading to release of marginated pool of Nt
Term
causes of monocytosis
Definition
chronic inflammatory states (autoimmune and infectious) and malignancy
Term
eosinophilia casues
Definition
allergic reactions (type I HSR), parasitic, Hodgkin lymphoma
Term
basophilia causes
Definition
CML
Term
lymphocytis leukocytosis causes
Definition
viral infection (T cell hyperplasia) bordatella pertussis (produces lymphocytosis-promoting factor which blocks them from leaving the blood into LN)
Term
cases of infectious mononucleosis
Definition
EBV, less commonly CMV
Term
what areas of body EBV infects
Definition
oropharynx, liver and B cells
Term
consequences of T cell response with EBV
Definition
generalized LAD 2/2 T cell hyperplasia in lymph node; spelnomegaly 2/2 T cell hyperplasia in PALS and high WBC in blood
Term
monospot test
Definition
detects IgM that cross react with horse or sheep RBC (heterophile Ab), positive within 1 week of infection
Term
mono complications
Definition
inc risk for splenic rupture; rash if exposed to ampicillin; dormancy of virus in B cells leads to inc risk of recurrence and B cell lymphoma, esp if immunodeficient
Term
% blasts in acute leukemia
Definition
>20%
Term
acute presentation of acute leukemia
Definition
inc blasts "crowd- out" normal hematopoiesis resulting in anemia, thrombocytopenia or neutropenia
Term
blast cell characteristics
Definition
large, immature cells often with punched out nucleoli
Term
marker for ALL cells
Definition
TdT+
Term
associated with Down syndrome after age 5
Definition
ALL
Term
most common type of ALL
Definition
B-ALL
Term
B-ALL cell markers
Definition
TdT+, CD10, CD19 and CD20
Term
prophylaxis in B-ALL
Definition
scrotum and CSF
Term
T-ALL cell markers
Definition
TdT+, CD2-CD8; DO NOT EXPRESS CD10
Term
cytogenic abnormalities in B-ALL and prognosis
Definition
t(12,21) = good prognosis, in children
t(9,22) = poor prognosis; in adults
Term
presentation of T-ALL
Definition
thymic mass
Term
marker for AML
Definition
MPO
Term
auer rods
Definition
AML
Term
translocation in APL
Definition
t(15;17) - retinoid acid receptor on chromosome 17 to chromosome 15
Term
increased risk for DIC
Definition
APL because promyelocytes contain numerous primary granules
Term
treatment of APL
Definition
ATRA = all trans retinoid acid
Term
blasts infiltrate gums
Definition
acute monocytic leukemia
Term
associated with Down syndrome before age 5
Definition
acute megakaryoblastic leukemia
Term
presentation of myelodysplastic syndrome
Definition
cytopenias, hyper cellular bone marrow; abnormal maturation of cells and increased blasts <20%
Term
cell markers in CLL
Definition
CD5 and CD20
Term
smudge cells on blood smear
Definition
CLL
Term
small lymphocytic lymphoma
Definition
CLL involvement of lymph nodes
Term
complications of CLL
Definition
hypogammagolbulinemia (infection most common cause of death), autoimmune hemolytic anemia, transformation to diffuse large B cell
Term
Richter transformation
Definition
CLL transform to diffuse large B cell; enlarging lymph node or spleen
Term
marker for hairy cel leukemia
Definition
TRAP+
Term
clinical features of hairy cell leukemia
Definition
splenomegaly (accumulation of hairy cells in red pulp) and "dry tap" in bone marrow aspiration
Term
hairy cell leukemia treatment
Definition
2-CDA (cladribine)= ADA inhibitor bc adenosine accumulates in neoplastic B cells
Term
ATLL virus
Definition
HTLV-1
Term
clinical features of ATLL
Definition
rash, generalized LAD with hepatosplenomegaly and lytic bone lesions with hypercalcemia
Term
mycosis fungoides abscesses
Definition
Pautrier = aggregates of neoplastic cells in epidermis
Term
sezary syndrome
Definition
lwhen mycosis fungicides involves blood; ymphocytes with cerebriform nuclei on blood smear
Term
complications of myeloproliferative disorders
Definition
increased risk for hyperuricemia and gout due to high turnover; progression to marrow fibrosis or transformation to acute leukemia
Term
translocation in CML
Definition
t(9;22)= philidelphia chromosome = bcr-abl fusion with increased tyrosine kinase activity
Term
CML treatment
Definition
imatinib
Term
how CML distinguished from leukemia reaction
Definition
CML = neg LAP stain, inc basophils, t(9;22)
leukemoid rxn = LAP+, no inc basophils or t(9;22)
Term
mutation in polycythemia vera
Definition
JAK2 Kinase
Term
symptoms of polycythemia vera
Definition
blurry vision and HA, inc risk of venous thrombosis, flushed face due to congestion, itching especially after bathing (histamine release)
Term
treatment of polycytemia vera
Definition
phlebotomy, second line = hydroxyurea
Term
how distinguish PV from reactive polycythemia
Definition
PV = EPO decreased and SaO normal
reactive = huh altitude/lung disease so SaO low and EPO inc; if due to ectopic EPO then SaO normal
Term
essential thrombocythemia mutation
Definition
JAK2 kinase
Term
symptoms of essential thrombocythemia
Definition
inc risk of bleeding and/or thrombosis; no gout
Term
cause of marrow fibrosis in myelofibrosis
Definition
megakaryocytes produce excess platelet-derived growth factor
Term
clinical features of myelofibrosis
Definition
splenomegaly due to extreamed hematopoiesis, leukoerythroblstic smear (tear drop RBC, nucleated RBC, immature granulocytes) inc risk of infection, thrombosis and bleeding
Term
painful vs painless LAD
Definition
painful = draining a region of acute infection
painless = chronic inflammation, metastatic carcinoma or lymphoma
Term
follicular hyperplasia is associated wth
Definition
rheumatoid arthritis and early stages of HIV
Term
paracortex hyperplasia
Definition
viral infection (mono)
Term
hyperplasia of sinus histiocytes
Definition
drainig a tissue with CA
Term
small sized NHL
Definition
follicular, mantle cell, marginal zone, and small lymphocytic
Term
intermediate NHL
Definition
burkitt
Term
large cell NHL
Definition
diffuse large B cell lymphoma
Term
translocation in follicular lymphoma
Definition
t(14;18) = bcl2 on chromosome 18 to Ig heavy chain on chromosome 14 -> over expression of bcl2 which inhibits apoptosis
Term
treatment for follicular lymphoma
Definition
rituximab = anti-CD20 Ab
Term
how to distinguish follicular lymphoma from follicular hyperplasia
Definition
lymphoma = disruption of normal architecture, lack of tangible body macrophages in germinal centers, bcl2 expression, monoclonality
hyperplasia = maintained, tangible body macrophages, no bcl2, polyclonl
Term
mantle cell translocation
Definition
t(11;14) = cyclin C2 on chromosome 11 to Ig heavy chain on chromosome 14 -> promotes G1'S transition in cell cycle
Term
associations of marginal zone lymphoma
Definition
hashimoto's, sjogren, H pylori gastritis
Term
MALToma
Definition
marginal zone lymphoma in mucosal sites that may regress with H pylori tx
Term
lymphoma with EBV
Definition
burkit
Term
african vs sporadic burkitt lymphoma
Definition
african = jaw
sporadic = abdomen
Term
Burkitt translocation
Definition
t(8;14) = c myc on chromosome 8 to Ig heavy chaing on 14 -> promotes cell growth
Term
burkitt histology
Definition
high mitotic index and "starry sky" appearance on microscopy
Term
most common form of NHL
Definition
diffuse large B cell
Term
causes of diffuse large b cell
Definition
sporadically or from low-grade lymphoma (follicular)
Term
RS cells
Definition
hodgkin lymphoma with multi lobed nuclei and prominent nucleoli (owl eyed)
Term
cytokines of RS cells
Definition
results in B symptoms (fever, chills, and night sweats), attract reactive lymphocytes, plasma cells, macrophages and eosinohpils; may lead to fibrosis
Term
subtypes of hodgkin lymphoma
Definition
nodular sclerosis, lymphocyte-rich, mixed cellularity, lymphocyte depleted
Term
most common HL subtype
Definition
nodular sclerosis
Term
nodular sclerosis subtype
Definition
enlarging cervical or mediastinal lymph node in young adult female; divided by bands of sclerosis and RS cell in lake-like spaces
Term
best prognosis of HL subtype
Definition
lymphocyte rish
Term
HL subtype with abundant eosinophils
Definition
mixed cellularity
Term
most aggressive HL subtype
Definition
lymphocyte-depleted
Term
most common primary malignancy of bones
Definition
multiple myeloma
Term
cytokine with multiple myeloma
Definition
IL6 = stimulates plasma cell growth and immunoglobin production
Term
clinical features of multiple myeloma
Definition
bone pain with hypercalcemia, elevated serum protein, inc risk of infection, rouleaux, primary amyloidosis, proteinuria
Term
cause of bone pain in MM
Definition
plasma cells activate RANK on osteoclasts leading to bone destruction; punched out lesions
Term
cause of elevated serum protein in MM
Definition
produc IG -> M spike on serum electrophoresis 2/2 IgG or IgG
Term
cause of rouleaux in MM
Definition
inc serum proein decreases charge between RBCs
Term
bence jones protein
Definition
free light chain in MM
Term
MGUS
Definition
M spike but other features of MM are absent; common in elderly; 1% with MGUS develop multiple myeloma each year
Term
waldenstrom macoglobulinemia
Definition
IgM production
Term
clinical features of waldenstrom
Definition
generalized LAD, no lytic bone lesions, M spike, visual and neuro dficites (IgM causing hyperviscosity), bleeding
Term
characteristics of langerhans cells
Definition
birbeck granules; CD1a+ and S100+
Term
letterersiwe disease
Definition
malignant; rash infant <2 y/o; rapidly fatal; multiple organs involved
Term
eosinohplic granuloma
Definition
benign langerhands; adolescent with pathologic fracture; mixed inflammatory cells on biopsy with langerhans
Term
hand-schuller-christian disease
Definition
malignant langerhans, scalp rash, lytic skull defects, DI and exopthalmos in child
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