Hematology

Question 1

scalp rash, skull defects, ear ache

Answer 1

Langerhans Cell Histiocytosis

dendritic cells in skin derived from marrow monocytes

Question 2

kid, TdT pos… anemia, thrombocytopenia, leukocytosis

Answer 2

ALL

often in kids, assoc with Down Syndrome

Question 3

more common ALL

Answer 3

B-ALL (CD10,19,20)

T-ALL would have CD2-8 and can present as lymphoma in teens (mediastinal mass)

Question 4

patho of acute leukemia

Answer 4

> 20% blasts in marrow lead to crowding out pancytopenia w/ inc WBC… blasts have punched out nucleoli
basically, suppression of hematopoiesis

Question 5

APL complication

Answer 5

DIC

Question 6

normocytic, normochromic anemia, thrombocytopenia, neutropenia, and leukocytosis of myeloblasts

Answer 6

AML

Question 7

anemia, thrombocytopenia, auer rods in cells, gum bleeding, DIC

Answer 7

APL

senescent cells degranulate -> DIC

Question 8

AML precursor

Answer 8

myelodisplastic syndrome

Question 9

pancytopenia, anemia, normochromic hypochromic RBCs…smear with sideroblasts & inc myeloblasts

Answer 9

myelodisplastic syndrome

Question 10

common cause of myelodisplastic syndrome

Answer 10

chemo

clonal SC defect -> lots more hyperplasia in marrow and fewer elements in blood

Question 11

anemia & leukocytosis (small lymphocytes) w/ inc smudge cells

Answer 11

CLL
rearrangement of IgG light chain gene activates bcl oncogene (no apoptosis)
folks over 50

Question 12

translocation in APL

Answer 12

RAR (retinoic acid receptor) -> it fuses with PML

treat with ATRA to cause blasts to mature

Question 13

prolif in CLL

Answer 13

small, naive B cells

Question 14

CLL + lymph node involvement

Answer 14

small lymphocytic lymphoma

Question 15

CLL change to diffuse large B cell lymphoma

Answer 15

Richter syndrome

Question 16

tartrate-resistant acid-phosphatase activity & splenomegaly

Answer 16

Hairy Cell leukemia

Question 17

rash/plaques with atypical lymphocytic infiltrate in dermis of CD4+ cells

Answer 17

Mycoses Fungoides
cutaneous T cell lymphoma of CD4 T cells
can spread to blood = Sezary Syndrome

Question 18

splenomeg, leukocytosis, t(9;22), hypercellular marrow of WBC precursors

Answer 18

CML

Question 19

oncogene in CML

Answer 19

abl -> bcr/abl fusion = constitutive tyrosine kinase

Question 20

leukocytosis of maturing neutrophils with basophilia and eosinophilia, inc platelets and t(9;22)

Answer 20

CML

leukocytosis over full range of myeloid maturation

Question 21

splenomeg w/o lymphadenopathy; inc Hb, WBC and platelets and depletion of iron stores

Answer 21

PV

Question 22

atypical megakaryocytes & marrow fibrosis w/ splenomeg -> sepsis

Answer 22

myelofibrosis

PDGF -> marrow fibrosis -> splenomeg, infxns and tear-drop RBCs

Question 23

inc platelets & megakaryocytes but no marrow fibrosis

Answer 23

Essential Thrombocythemia

lots of thrombosis & hemorrhage

Question 24

fever, chills, enlarged supraclavicular node, Reed Sternberg cells

Answer 24

Hodgkin Lymphoma

Question 25

most common subtype of hodkin lymphoma

Answer 25

Nodular Sclerosis - collagenous fibrosis & lacunar RS cells (this is why cervical nodes enlarge)

Question 26

proliferation derived from B cell germinal centers with lots of Bcl-2

Answer 26

non-hodgkin follicular lymphoma

Question 27

t(14;18) w/ IgH & Bcl-2

Answer 27

non-hodkin follicular lymphoma

Question 28

follicular (cortex) lymph node hyperplasia

Answer 28

B cells

Question 29

paracortex lymph node hyperplasia

Answer 29

T cells

Question 30

expanding jaw... surface IgM & B cell markers

Answer 30

Burkitt lymphoma (extranodal tumors)

Question 31

Burkitt oncogene

Answer 31

c-myc

Question 32

HIV+, EBV+, enlarged node

Answer 32

Large B Cell lymphoma (common in AIDS)

Question 33

neoplastic prolif with lymphocytic infiltrate arising in context of chronic infxn

Answer 33

Marginal Zone lymphoma | MALT associated

Question 34

large nodes with cytoplasmic IgM and plasmacytoid lymphocytes

Answer 34

waldenstrom disease (lymphoplasmacytic lymphoma)

Question 35

lytic lesions, 18% plasma cells, anemia, thrombocytopenia

Answer 35

``` multiple myeloma (plasma cell myeloma) plasma cells infiltrate marrow ```

Question 36

extramedullary complications of multiple myeloma

Answer 36

renail failure via amyloid nephropathy and fractures | -also risk of infxn, rouleaux formation

Question 37

kid with pinpoint hemorrhages, virus 1 week ago, dec platelets, inc megakaryocytes... 2 month later normal platelets

Answer 37

ITP (Abs against platelets) | -acute in kids, chronic in adults

Question 38

SLE, petechiae, fatigue, lympadenpathy, splenomeg, normocytic anemia, thrombocytopenia

Answer 38

Ab-mediated platelet destruction if reticulocytes were high, it would be hemolytic anemia

Question 39

EBV, tingling fingertips, rouleax formation

Answer 39

Immune hemolytic anemia -> cold agglutinins and autoAbs against RBC membrane most hemolytic anemias are warm agglutinins mediated by IgG

Question 40

vag bleeding, jaundice, purpura, dec Hb, 8% retics, dec platelets and schistiocytes

Answer 40

TTP (microangiopathic hemolytic anemia)

Question 41

burns, anemia, thrombocytopenia & purpura, inc fibrin split products

Answer 41

microangiopathic hemolytic anemia from turbulent blood flows caused by burn

Question 42

ecchymosis and easy bruising with low factor 8

Answer 42

hemophilia A

Question 43

red urine in morning, anemia, low serum iron, inc reticulocytes, inc lysis upon acidification

Answer 43

paroxysmal nocturnal hemoglobinuria

Question 44

pallor of oral mucosa & glossitis, macrocytic anemia and atrophic gastritis... megaloblastic erythroid maturation & hypercellularity

Answer 44

pernicious anemia | Abs against parietal cells and intrinsif factor lead to b12 deficiency so impaired DNA synth

Question 45

petechiae w/ dec Hb, platelets and WBC and hypocellular marrow

Answer 45

aplastic anemia

Question 46

heavy menstrual bleeding w/ microcytic, hypochromic cells and low plasma iron sats

Answer 46

iron def anemia. also dec. ferritin

Question 47

jaundice, splenomeg, inc osmotic fragility, neg coombs

Answer 47

hereditary spherocytosis | defects in RBC cytoskeleton

Question 48

spherocytosis inc risk for

Answer 48

cholethiasis due to extravasc hemolysis

Question 49

3 cytoskel proteins that can be mut in spherocytosis or elliptocytosis

Answer 49

spectrin, ankryn and protein c

Question 50

infant w/ splenomeg & jaundice, mycrocytic hypochromic anemia w/ mostly HbF

Answer 50

Beta thallasemia | target cells on smear

Question 51

why splenomeg in so many conditions that cause anemia?

Answer 51

extramedullary hematopoeisis

Question 52

alpha 2 beta 2

Answer 52

HbA

Question 53

alpha 2 gamma 2

Answer 53

HbF

Question 54

alpha 2 delta 2

Answer 54

HbA2 (inc in heterozygous Beta thall)

Question 55

PV inc risk for

Answer 55

cerebrovascular incident due to hyperviscosity

Question 56

common complication of sickle cell

Answer 56

ischemia also autosplenectomy, inc risk of encapsulated bugs like strep pneumoniae

Question 57

what would cause a rapid drop in Hb with low retics in many anemic disorders

Answer 57

parvovirus B19 infxn

Question 58

TB, microcytic anemia, low serum iron, inc serum ferritin

Answer 58

anemia of chornic disease

Question 59

alcoholic, spiny projections on smear

Answer 59

acanthocytosis via liver disease