chapter 13- WBCs Flashcards
what is the CD34+ cell
hematopoietic stem cell
what is the term for low WBC count
leukopenia
what is the term for high WBC count
leukocytosis
what is neutropenia classically seen with
drug toxicity and severe infection
what can cause the eft shift with neutrophilic leukocytosis
bacterial infection and tissue necrosis because slightly immature neutrophils may be released (decreased Fc receptors)
how does high cortisol state cause neutrophilic leukocytosis
because it causes demargination
what is produced in hodgkin lymphoma that can lead to eosinophilia
increase production of IL-5
what is the lymphocytic leukocytosis associated with infectious mononucleosis comprised of
reactive CD8+ T cells
what is the presentation result of neoplastic proliferation of blasts
acute presentation with anemia, thrombocytopenia or neutropenia
what are lymphoblasts positive for in the nucleus
tDt
what are myeloblasts positive for
MPO
what is the condition classified as neoplastic accumulation of lymphoblasts
ALL
who does ALL most commonly arise in
kids
at what age is ALL associated with down syndrome
after age 5
what is the most common ALL and what does it express
B-ALL; CD10, CD19, CD20
what is the most common translocation in kids with B-ALL
t (12:21)
what is the most common translocation in B-ALL
t (9:22)
how does T-ALL present
mediastinal mass in teenagers
what is the term for neoplastic accumulation of myeloblasts
AML
who does AML typically present in
older adults (50-60yo)
what translocation is acute promyelocytic leukemia characterized by
t(15:17)
what receptor is disrupted in acute promyelocytic leukemia
RAR receptor
what do the promyelocytes in acute promyelocytic leukemia contain numerous of and what does this pose a risk for
numerous Auer rods; risk for DIC
what do the blasts in acute monocytic leukemia characteristically infiltrate
gums
what disorder is acute megakaryoblastic leukemia associated with
down syndrome before the age of 5
what is the term for neoplastic proliferative of naive B cells that coexpres CD5 and CD20
CLL
what should you think of when you see smudge cells
CLL
what are some complications of CLL
hypogammaglobulinemia, autoimmune hemolytic anemia, transformation to diffuse large B cell lymphoma
what is the term for neoplastic proliferation of mature B cells
hairy cell leukemia
what are the cells in hairy cell leukemia positive for
TRAP
what does hairy cell leukemia have excellent response to
2-CDA
what typically causes the death in individuals with hair cell leukemia
adenosine accumulates to toxin levels in neoplastic B cells
what is the term for neoplastic proliferation of mature CD4+ T cells
ATLL
what is ATLL associated with
HTLV-1 (japan and caribbean)
what is the clinical presentation associated with ATLL
rash, generalized LAD with HSM, lytic bone lesions with hypercalcemia
what is the term for aggregates of neoplastic T cells in the epidermis and what disease is it associated with
paltrier micrabscesses; mycosis fungoides
what is it called when cells of mycosis fungicides spread to the blood
sezary syndrome
what are the characteristic lymphocytes in sezary syndrome
cerebriform nuclei
what is chronic myeloid leukemia driven by
t (9:22)
are CML granulocytes LAP positive or negative
negative
what is the term for neoplastic proliferation of mature myeloid cells, especially RBCs
polycythemia Vera
what is also increased besides RBCs in my polycythemia vera
granulocytes and platelets
if someone is itching after bathing, have blurry vision and headache and a flushed face, what disorder might you consider
polycythemia vera
what mutation in polycythemia associated with
JAK2 kinase mutation
what is the term for neoplastic proliferation of mature myeloid cells, especially platelets
essential thrombocythemia
what cells are increased in essential thrombocythemia
RBC and granulocytes
what mutation is associated with essential thrombocythemia
JAK2 kinase
what is the term for neoplastic proliferation of mature myeloid cells, especially megakaryocytic
myelofibrosis
what mutation is associated with myelofibrosis
JAK2 kinase
what is produced in excess in myelofibrosis
PDGF
what is the clinical presentation of follicular lymphoma
late adulthood with painless LAD
what is follicular lymphoma driven by
t (14:18)
what can follicular lymphoma progress to as a complication
diffuse large B cell lymphoma
how does large B cell lymphoma present
enlarging LN
what should you think of with follicular hyperplasia
tangible body macrophages in GC
how is mantle cell lymphoma presented clinically
late adulthood with painless LAD
what is mantel cell lymphoma driven by
t(11:14)
what is highly over expressed in mantle cell lymphoma
cyclin D1
what is the clinical presentation of Burkitt lymphoma
extra nodal mass in kids or young adults
what does the african form of burkitt lymphoma typically involves
jaw
what does the sporadic form of burkitt lymphoma typically involve
abdomen
what translocation is common in burkitt lymphoma
c-myc (chr 8); t(8:14)
what lymphoma has starry sky appearance
Burkitt lymphoma
what is the most common form of NHL
DLBCL
what should you think of with reed-sternberg cells
hodgkin lymphoma
describe the nucleus in reed sternberg cells
CD15+, CD30+; owl-eyed nucleus
what subtype of hodgkin lymphoma has the best prognosis
lymphocyte rich
what subtype of hodgkin lymphoma is associated with abundant eosinophils
mixed cellularity type
what subtype of hodgkin lymphoma has the worst prognosis and is seen in the elderly or HIV+
lymphocyte depleted
how do the RS cells present in nodular sclerosis type of hodgkin lymphoma
lake-like spaces (lacunar cells)
what is the term for malignant proliferation of plasma cells in bone marrow
multiple myeloma
what is the most common primary malignancy of bone
multiple myeloma
describe the Xray seen in multiple myeloma
lytic punched out lesions seen on X-ray, especially in vertebrae and skull
what is the most common cause of death in multiple myeloma
infection
what should you think of with rouleaux formation on blood smear
multiple myeloma
what is primary AL amyloidosis a consequence of
multiple myeloma
how is the proteinuria seen in primary AL amyloidosis excreted
as Bence-jones proteins
what is the term or B cell lymphoma with monoclonal IgM production
waldenstrom macroglobulinemia
what is the clinical presentation of waldenstrom macroglobulinemia
generalized LAD; lytic bone lesions are NOT present; increased serum protein with M spike; visual and neurological deficits
what should you think of with birbeck/tennis racket lesions on EM
langerhans cell histiocytosis
how is letterer-siwe disease presented
skin rash and cystic skeletal defects in an infant
what is the term for benign proliferation of langerhans cells in bone
eosinophilic granuloma
what is the presentation of eosinophilic grandma
pathologic fracture in adolescents; skin NOT involved
what is the presentation of Hand-schuller-christian disease
scalp rash, lytic skull defects, DI, exophthalmos in a kid
What is the chief site of blood formation until shortly before birth
Liver
What is the term for abnormal release of immature precursors into the peripheral blood
Leukoerythroblastosis
What is the normal range for adult granulocytes
40-70%
What is the most common cause of agranolocytosis in neutropenia
Drug toxicity
What congenital condition has impairment of granulocytic differentiation
Kostmann syndrome
What is the most common consequence of agranulocytosis
Infections
What is the term for patches of dilated ER that appear as sky-blue cytoplasmic puddles seen with leukocytosis
Dohle bodies
What is the term for coarser and darker than normal neutrophilic granules seen in leukocytosis
Toxic granules
What can cause leukocytosis due to increased release from marrow stores
Endotoxemia, infection, hypoxia
What can cause decreased margin action leading to leukocytosis
Exercise, catecholamines
How does acute nonspecific lymphadenitis present
Painful red abscess
What is acute non-specific lymphadenitis in the axillary or inguinal regions most often caused by
Infections in extremities
What are some causes of follicular hyperplasia
Rheumatoid arthritis, toxoplasmosis, early stages of HIV
What is another name for reticular hyperplasia
Sinus histiocytosis
What are myodysplastic myeloid neoplasms associated with
Ineffective hematopoiesis and resultant peripheral blood cytopenias
What do Bloom and Fanconi increase the risk of
Acute leukemia
What is the origin of most lymphoid neoplasms
B cell origin
What is the primary T cell associated with CD1
Thymocytes and langerhans cells
What are the primary T cells associated with CD3
Thymocytes, mature T cells
What are the primary B cells associated with CD10
Pre-B cells and germinal center B cells
What are the primary B cells associated with CD19
Pre B cells and mature B cells but not plasma cells
What is the EBC receptor
CD21
What B cells is CD23 primarily associated with
Activated mature B cells
What is the primary cell associated with CD14
Monocytes
What are the primary cells associated with CD15
Granulocytes’ Reed-Sternberg cells and variants
What cells are associated with CD64
Mature myeloid cells
What is the most common cancer of childhood
ALL
What is the peak incidence for B-ALL
Age 3
What is mutation in T-ALLs
NOTCH1
Are lymphoblasts myeloperoxidase positive or negative
Negative
What is the specialized DNA polyermase that is expressed only in pre-B and pre-T lymphoblasts and is positive in 95% of classes in ALL
TdT
What do pre-B cells usually epxress
CD19, PAX5, CD10
What is the most common leukemia of adults in western world
CLL/SLL
What are human CLL cells dependent on
BTK pathway
What is the immunophenotype in CLL,SLL
CD19+, CD20+, CD23+, CD5+
What is the most common form of indolent NHL
Follicular lymphoma
What is follicular lymphoma strongly associated with chromosomal translocations involving
BCL2
What is disregulated in diffuse large B cell lymphoma
BCL6
What is primary effusion lymphoma associated with
KSHV/HHV-8
What translocation is involved in Burkitt lymphoma
MYC gene on chromosome 8
What is the immunophenotype of Burkitt lymphoma
Express IgM, CD19, CD20, CD10, BCL6; almost always FAILS to express BCL2
What is the most common and deadly plasma neoplasm
Multiple myeloma
What heavy chain gene rearrangement is common in multiple myeloma
Chromosome 14q32
What is the most common plasma cell dyscrasia
MGUS
What is the genotype on large granular lymphocytic leukemia
Point mutations in STAT3
What mutations are associated with lymphoplasmacytic lymphoma
Acquired mutations in MYD88
When you see Russell bodies or ditched bodies, what should you think of
Lymphoplasmacytic lymphoma
What infectio. Must be present to get adult T-cell leukemia/lymphoma
HTLV-1
What does HTLV-1 encode
Tax
What should you think of with cloverleaf or flower cells
Adult T cell leukemia or lymphoma
What is the triad associated with felty syndrome
Rheumatoid arthritis, splenomegaly, neutropenia
What presentation is most common with extranodal NKT-cell lymphoma
Destructive nasopharyngeal mass
What are the classic type of Hodgkin lymphoma positive for
PAX5, CD15, CD30
What type of Hodgkin lymphoma is NOT associated with EMV
Lymphocyte predominance
What disease should you think of with Auer rods
AML
What type of syndromes should you think of with pseudo-pelger-Huet cells and pawn ball megakaryocytes
Disordered differentiation in myelodysplastic syndrome
What is the common mutation in chronic myelogenous leukemia
BCR-ABL fusion gene
What is the mutations in essential thrombocytopenia
JAK2 and MPL point mutations
What is the mutation in systemic mastocytosis
KIT point mutations
What should you think of with sea-blue histiocytes
CML
How is essential thrombocytosis separated from PCV and primary myelofibrosis
Based on absence of polycythemia and marrow fibrosis, respectively
What disorder is erthromelalgia seen in
Essential thrombocytosis
What is the term for throbbing and burning of hands and feet caused by occlusion of small arterioles by platelet aggregation
Essential thrombocytosis
What is the chief pathology feature of primary myelofibrosis
Extensive deposition of collagen in marrow by non-neoplasticism fibroblasts
How does primary myelofibrosis come to attention
Because of progressive anemia and splenomegaly, which produces sensation of fullness in LUQ
What is the hand-Schiller-Christian triad associated with
Eosinophilic granuloma
What is pulmonary langerhans cell histiocytosis most common in
Adult smokers
What do neoplastic langerhans cells express
Both CCR6 and CCR7
Where do most thymomas arise
Anterior superior mediastinum