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WBC differential from highest to lowest.
NLMEB
Nphils
Lymphocytes
Monocytes
Ephils
Bhpils
Spectrin:
Role
Allow for biconcave shape of RBCs
What is the source of energy for RBCs?
Glucose and only glucose.
Anisocytosis vs Poikilocytosis
Anisocytosis = varying sizes
Poikilocytosis = varying shapes
What are causes of polycythemia?
Renal Cell Carcinoma
HCC
Pheochromocytoma
Hemangioblastoma
Chronic hypoxia (COPD, high altitude, obstructive sleep apnea)
Down Syndrome (65% at birth)
Polycythemia vera (excess RBCs despite low EPO)
What is the lifespan of platelets?
Where are they stored?
PLT lifespan = 8-10 days
Stored in spleen
This cell mediates type I hypersensitivity reactions.
Mast cells
Cromolyn:
MOA
Use
Prevents mast cell degeneration; used to tx asthma
What are the causes of eosinophilia?
D NAAACP
Drugs
Neoplasm
Allargy, Asthma (Churg-Straus)
Addison's (adrenal insuff)
AIN
Collagen vasc dz
Parasites (Loeffler's ephilic pneumonitis due to Ascaris lumbricoides)
Type of phagocyte in:
Brain
Liver
Joints
Brain-microglia
Liver-Kupffer cell
Joints-A cells
B cells:
CD Markers
Role
CD19, CD20

When antigen encountered, diff into plasma cells and produce antibodies.
Exhibit memory.
Can function as APC via MHC II
What organelle is most prominent in plasma cells?

Why?
Plasma cell needs to make a lot of protein which is to be exported (immunoglobulins), therefore, has abundant RER.
T lymphocytes:
CD Markers
Th: CD3, CD4
Tc: CD3, CD8
What clotting factor initiates the extrinsic pathway?
VII
What clotting factor initiates the intrinsic pathway?
XII
Which coagulation pathway forms kallikrein and bradykinin?
Intrinsic PW (XII)
Hemophilia A vs B:
General
Hemophilia A: Ate--Eight--deficiency in VIII

Hemophilia B: Benign--Nine--deficiency in IX
What is the effect of IV citrate on cogaulation?
IV citrate = calcium binder
Calcium is essential to coagulation

Dec'd available Ca2+-->Dec'd coaguln
What is the role of antithrombin?
AKA?
Antithrombin = Factor IIIa (activated)

Factor III inhibits thrombin (factor IIa), IX, X, XI, XII
What is the result of a vitamin K deficiency on coagulation factors?
Dec'd synthesis of factors II, VII, IX, X, Protein C, protein S
Heparin:
MOA
Use
AE
Antitode
Cofactor for activation of antithrombin (IIIa); decreases thrombin, and Xa (short half-life)

Use: Immediate anticaoag for PE, stroke, acute coronary syndrome, MI, DVT

Safe in pregnancy

AE: HIT, osteoporosis

Antidote: Protamine sulfate (positively charged molecule that will bind negatively charged heparin)
Enoxaparin:
Drug Class
MOA
Use
AE
LMW Heparin; acts more on Xa; better avaoilability and longer half-life

RIsk HIT, not easily reversible
Heparin-induced thrombocytopenia:
Pathophys
Tx
Heparin binds PLT factor IV-->leads to Ab production-->activates PLTs leading to their clearance and resulting in thrombocytopenic, hypercoag state (it's paradoxical)


Tx: Lepirudin or bivalirudin in addition to warfarin
Leiprudin:
MOA
Use
Directly inhibit thrombin; used as alternative to heparing for anticoagulating pts with HIT
Warfarin:
MOA
Use
Labs to monitor it
AE
Antidote
Interferes with synthesis of vitamin K-dependent clotting factors II, VII, IX, X, protein C, protein S (DiSCo started in 1972--10(X),9(IX),7(VII),2(II))

Has effect on extrinsic PW, increasing PT (WEPT); use INR to standardize PT values

AE: TERATOGEN, bleeding

Antidote: IV Vitamin K, fresh frozen plasma
What are the effects of bradykinin?
Inc'd vasodilation
Inc'd perm
Inc'd pain
Angioedema--swelling from lips to larynx (DANGEROUS)
What thrombogenic factors are located inside vascular endothelial cells?
vWF
Thromboplastin
tPA
PGl2
vWF:
Cells that synthesize it
Functions
Signs of deficiency
Made by endothelial cells and megakaryocytes

Fn:
Complexes with and stabilizes Factor VIII (deficiency-->inc'd PTT)
PLT adhesion to vessel wall and to other platelets (deficiency-->Inc'd bleeding time)
Describe the steps required to achieve platelet adhesion and aggregation.

Begin with endothelial cell damage.
Endothelial cell damage-->Release vWF-->binds exposed collagen

vWF binds GpIb on PLT (adhesion of PLT)

PLT is activated and now secretes ADP, PDGF, thromboxane A2 (vasoconstriction, PLT agg), Calcium (for coag pw), and thrombin (fibrinogen-->fibrin)

Aggregation of PLT via GpIIb/IIIa
How exactly does ASA affect the coagulation cascade?
Inhibit COX-->dec'd TXA2 synthesis and thus dec'd PLT aggregation
What is the effect of clopidogrel on the coagulation cascade?

What other drug has this same effect?
Clopidogrel and ticlopidine:
Irreversibly inhibit ADP receptors required to insert GpIIb/IIIa receptor into PLT cell membrane (thus, dec'd agg)
What is the effect of abciximab on the coagulation cascade?
Abciximab directly inhibit GbIIb/IIIa to dec PLT agg
What allows RBCs to change shape as they pass through vessels?
Spectrin
What are some of the different causes of polycythemia?
Excess EPO:
-RCC
-Pheo
-Hemangioblastoma
-Chronix hypoxia (COPD, OSA)

Polycythemia vera
Down Syndrome
What coagulation factor is deficient in hemophilia A?
VIII
What coagulation factor is deficient in hemophilia B?
IX
What clotting factors require vitamin K for synthesis?
X, IX, VII, II
C, S

DiSCo started in 1972
What are the treatments for overdose of heparin and warfarin?
Heparin--protamine sulfate

Warfarin--FFP, vitamin K
What lab value is used to monitor the following medications: heparin, warfarin, enoxaparin?
Heparin: PTT
Warfarin: PT/INR
Enoxaparin: Xa
What is the treatment for heparin-induced thrombocytopenia?
Lepirudin or bivalirudin
Schistocyte vs Spherocyte::
Appearance
Cause
Schistocyte: helmet cell; seen in DIC, TTP/HUS, traumatic hemolysis

Spherocyte = round; hereditary spherocytosis, autoimmune hemolysis
Which RBC form:
Lead poisoning
Basophilic stippling
Which RBC form:
G6PD deficiency
Heinz bodies, bite cells
Which RBC form:
DIC
Schistocyte (helmet cell)
Which RBC form:
Abetalipoproteinemia
Acanthocytes (spur cell)--also seen in liver dz (acantho = spiny)
Which RBC form:
Asplenia
Howell-Jolly BOies
acanthocyte (spur cell): seen in liver dz, abetalipoproteinemia
Basophilic stippling--Baste the ox TAIL
Thalassemias
Anemia of chronid dz
Iron def
Lead poisoning
Hb C vs Hb S:
Structure
Hb C: alpha2-beta2; glu-->lys in beta chain

Hb S: alpha2-beta2: glu-->val in beta chain
Hg Bart vs Hb H:
Structure
Hg Bart: gamm-4; no alpha chains (severe alpha-thal)

Hb H: beta-4; no alpha chains (severe alpha-thal)
Target cells--due to increase in SA:V ratio from Fe def anemia (dec'd cell volume) or in obstructive liver dz (inc'd cell membrane)
Target cell
Thalassemia major--blood disorder caused by defect in beta-chain synthesis in Hgb. Note presence of target cells.
Fe def anemia; microcytosis and hypochromia can be seen
Sickle cell anemia--note sickled cells as well as anisocytosis, poikilocytosis, and nucleated RBCs
alpha-thalassemia:
Subtypes
–a–aa: silent carrier
– – -aa: alpha-thalasssemia trait
– – - –a: HbH
– – - – –: Hydrops fetalis (Hb Barts)
beta-thalasemia:
Subtypes
-b: beta-thal minor; usually asyx
- -: beta-thal major; severe anemia requiring blood transfusion (worry about secondary hemochromatosis), skeletal deformities
Lead poisoning:
Affected enzyme
Presentation
Treatment
Affected enzyme: Ferrochelatase, ALA dehydratase (aminolevulinic acid = ALA)

Microcytic anemia, GI and kidney dz, lead lines on gingivae and on epiphyses of long bones on x-ray
Basophilic stippling
Wrist, foot drop

Children-->MR
Adults-->HA, memory loss, demyelination

Tx:
Sucks to be a kid with lead poisoning-->Succimer

Adults-->Dimercaprol and EDTA
Sideroblastic anemia:
Pathophys
Treatment
Defect in heme synthesis due to X-linked defect in aminolevulinic acid synthase gene

Tx: pyridoxine (B6 tx)

Presents with ringed sideroblasts (with iron-laden mitochondria)
Transferrin:
Role
Transports iron in blood
Ferritin:
Role
Stored form of iron
Iron deficiency anemia vs Anemia of chronic disease:
Serum Fe
TIBC
Ferritin
Fe/TIBC
Iron def anemia
Fe: low
TIBC: high
Ferritin: low
Fe/TIBC: low low

Anemia of chronic dz:
Fe: low
TIBC: low
Ferritin: high (elevated in infections)
Fe/TIBC: normal or high
Hemochromatosis:
Serum Fe
TIBC/Transferrin
Ferritin
Fe/TIBC
Serum Fe: high
TIBC/Transferrin: low
Ferritin: high
Fe/TIBC: high high
Pregnancy/OCP:
TIBC/transferrin
Fe/TIBC
TIBC/transferrin high (dec'd transferrin)
Fe/TIBC low
Lead poisoning:
Serum Fe
TIBC/Transferrin
Fe/TIBC
Lead takes up iron's spot in heme synthesis so:
Serum Fe: high
TIBC/Transferrin: low
Fe/TIBC: high
Folate deficiency:
Findings
Causes
Hyperseg'd nphils
Glossitis
Inc'd homocysteine, nl MMA

Causes:
EtOH (malnutrition), malabsorption, impaired metabolism (MTX, trimethroprim), inc'd requirement (hemolytic anemia, pregnancy)
B12 deficiency:
Findings
Cause
Hyperseg'd nphils, glossitis, elevated homocysteine, ELEVATED MMA (methylmalonic acid)

Causes:
Insufficient intake (strict vegans)
Malabs (Crohn's dz)
Pernicious anemia
Diphyllobothrium latum (fish tapeworm)

NEURO SYX (B12 needed for FA synth--think myelin):
Peripheral neuropathy with sensorimotor dysfn
Posterior columns (vibration/proprioception)
Lateral corticospinal (spasticity)
Dementia
Normocytic, normochromic anemia:
Intravascular vs Extravascular causes
Intravascular:
Paroxysmal nocturnal hemoglobinuria
Autoimmune hemolytic anemia
Mechanical destruction (aortic stenosis, prosthetic valves)

Extravascular:
Hereditary spherocytosis
G6PD def
Pyruvate kinase def
Sickle Cell
HgC defect
Nonhemolytic, normocytic anemia:
Causes
Anemia of chronic dz
Aplastic anemia (destruction of myeloid cells)
Kidney dz (low EPO)
Anemia of chronic disease:
Pathophys
Inflammn-->inc'd hepcidin-->dec'd release of Fe from macs

Dec'd iron, dec'd TIBC, dec'd ferritin

Can become microcytic, hypochromic in long-standing dz
Aplastic anemia:
Findings
Pancytopenia characterized by severe anemia, neutropenia, thrombocytopenia

Normal cell morphology, but hypocellular bone marrow with fatty infiltration

Can be due to: radiation, drugs, alkylating agents, parvovirus, EBV, HIV, HCV, immune mediated; stem cell defect
What are the causes of megaloblastic anemia?
MAPLE
MTX
AZT
Phenytoin
Liver Dz
Ethanol
G6PD Deficiency:
Pathophys
X-linked
Defect in G6PD-->dec'd glutathione-->inc'd RBC susceptibility to oxidant stress
What are the complications of Sickle Cell Disease?
-Aplastic crisis (due to parvovirus B19 infection)
-Autosplenectomy-->inc'd risk infection w/encapsulated organisms; functional splenic dysfn occurs in early childhood

-Salmonella osteomyelitis

-Pain crisis (vaso-occlusive)

-Renal papillary necrosis (due to low O2 in papilla) and microhematuria (medullary infarcts)

Splenic sequestration crisis (infarcts)
Cause of anemia:
Microcytic anemia + swallowing difficulty + glossitis
Plummer-Vinson Syndrome (which is assocd w/Fe def anemia)
Cause of anemia:
Microcytic anemia + > 3.5% HbA2
beta-thal minor
Cause of anemia:
Megaloblastic anemia not correctable by B 12 or folate
Orotic aciduria
Cause of anemia:
Megaloblastic anemia along with peripheral neuropathy
B12 def
Cause of anemia:
Microcytic anemia + basophilic stippling
Lead poisoning
Cause of anemia:
Microcytic anemia reversible with B6
Sideroblastic anemia
Cause of anemia:
HIV positive patient with macrocytic anemia
Zidovudine
Cause of anemia:
Normocytic anemia + red urine in the morning
Paroxymal Nocturnal hemoglobinuria
Cause of anemia:
Normocytic anemia and elevated creatinine
Chronic kidney dz resulting in low EPO levels
Cold agglutinins:
Pathophys
Causes
Cold ice cream? MMMMMM

Nearly always IgM; antibodies against RBCs interact at low temp (4ºC): IgM-->RBC antigen-->compliment fixation-->MAC lysis

Occurs with Mycoplasma pneumoniae, EBV, some malignancies
Warm agglutinins:
Pathophys
Causes
Warm weather is GGGreat

Antibodies react against RBC antigens at body temp, nearly always IgG

Seen in:
Viruses--EBV, HIV
SLE
Malignancies (CLL, NHL)
Congenital Immune Abnlts
Direct vs Indirect Coomb's:
General
Uses
Direct Coomb's (DAT):
Prepared Ab's added to pt's washed RBC to detect presence of immunogllobulins already present on RBC
(+) in Hemolytic Dz of Newborn, Drug-induced hemolytic anemia, Hemolytic transfusion rxns

Indirect Coomb's:
Pt's serum incubated w/normal RBC to detect presence of Ab's

(+) when ab's present to foreign blood (used to test blood prior to transfusion), screening for maternal Ab's to a fetus' blood
Microangiopathic anemia:
Pathophys
Findings
RBCs damaged when passing thorugh obstructed or narrowed vessel lumina

Seen in DIC, SLE, malignant HTN, TTP

Schistocytes seen on blood smea due to mechanical destruction of RBCs
Macroangiopathic anemia:
Pathophys
Prosthetic heart valves and aortic stenosis cause hemolytic anemia 2º to mechanical destruction
What is the rate-limiting step of heme?
aminolevulinic acid synthase (Glycine + succinyl-CoA-->aminolevulinic acid)
Acute intermittent porphyria:
Presentation
Affected enzyme
Accumulated substrate
Painful abdomen with normal CT (due to neuro dysfn)
Port wine urine
Polyneuropathy
Psych disturbances
Precipitated by drugs

Acute: think acute abdomen (pain) and acute psychoses

Affects Porphobilinogen deaminse (uroporphyrinogen-I-synthase)-->results in porphobilinogen, ALA, uroporphyrin buildup in urine
Porphyria Cutanea Tarda:
Presentation
Affected enzyme
Accumulated substrate
Blistering cutaneous photosensitivity; most common porphyria
Also associated with hypertrichosis, facial hyperpigmentation, elevated LFTs, HCV, EtOH

Enzyme affected: uroporphyrinogen decarboxylase

Buildup of uroporphyrin in urine
Hemophilia A/B:
Effect on bleed time
Presentation
Both will increase PTT; PT will be normal because hemophilia only affects INTRINSIC PW

Presents with hemarthroses, easy bruising
Vitamin K deficiency:
Effect on bleed time
Presentation
Inc'd PT and PTT (general coagulation effect--both extrinsic and intrinsic pw's)
Platelet disorders:
General presentation
Microhemorrhage-->mucous membrane bleeding, epistaxis, petechiae, purpura, dec'd platelet count
Bernard-Soulier Disease:
Effect on platelet count
Effect on bleed time
Pathophys
Dec'd PC
Inc'd BT

Dec'd GpIb-->defect in platelet-to-collagen adhesion; can't form platelet plug
Glanzmann's thrombasthenia:
Effect on platelet count
Effect on bleed time
Pathophys
Normal PC (no thrombocytopenia)
Inc'd BT

Dec'd GpIIb/IIIa-->defect in platelet-to-platelet aggregation; defect in PLT plug formation

Blood smear shows no platelet clumping
Idiopathic thrombocytopenia purpura:
Effect on platelet count
Effect on bleed time
Pathophys
Treatment
Dec'd PC
Inc'd BT

anti-GpIIb/IIIa Ab's-->peripheral platelet destruction-->dec'd PLT survival

Inc'd # megakaryocytes

Tx: Steroids
Thrombotic thrombocytopenic purpura:
Effect on platelet count
Effect on bleed time
Pathophys
Dec'd PC
Inc'd BT

Deficiency of ADAMTS13 (vWF metalloprotease)-->dec'd degradation of vWF multimers

Large vWF multimers-->inc'd platelet agg and thrombosis

Labs: schistocytes, inc'd LDH
Neuro and renal syx, fever, low PLT, microangiopathic hemolytic anemia
vonWillebrand's disease:
Effect on platelet count
Effect on bleed time
Effect on PT/PTT
Pathophys
Normal PC, elevated BT, normal PT

Dec'd vWF-->nromal or elevated PTT 9depends on severity; vWF carries/protects factor VIII)

Leads to defect in intrinsic PW coag, defect in PLT plug formation (no platelet-to-collagen adhesion)

Mild but common
DIC:
Effect on platelet count
Effect on bleed time
Effect on PT/PTT
Pathophys
Labs
Dec'd PC
Inc'd BT
Inc'd PT/PTT

Widespread activation of clotting-->deficiency in clotting factors-->bleed state

Causes: STOP Making New Thrombi:
Sepsis, Trauma, Ob complications, Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion

Labs: schistocytes, inc'd fibrin split products (D-dimers), dec'd fibrinogen, dec'd FV, FVIII
What is the rate-limiting step in heme synthesis?
ALA synthase (aminolevulate)
What is the cause of ITP?
Anti GpIIb/IIIa-ab's-->PLT destruction
What is the defect in Bernard-Soulier disease?
Def of GpIb
What is the most common inherited bleeding disorder?
vW Dz
What are some of the hereditary syndromes of thrombosis?
Factor V Leiden mutation
Prothrombin gene mutation
Antithrombin III def
Prot C def
Prot S def
Hematologic tests/findings:
Ham's test
Used to dx Paroxysmal Nocturnal Hemoglobinuria
Hematologic tests/findings:
DEB test
Used to dx Fanconi's anemia
Hematologic tests/findings:
Heinz bodies
G6PD def
Hematologic tests/findings:
D-dimers
Fibrin degradation products seen in DIC (or big clot like emboli, DVTs)
Hematologic tests/findings:
Basophilic stippling
BASt the ox TAIL
Thal
Anemia of chronic dz
Iron def anemia
Lead poisoning
Hematologic tests/findings:
Osmotic fragility test
Used to dx hereditary spherocytosis
Hematologic tests/findings:
(+) ristocetin test
Used to dx von Willebrand's Dz
Leukemia vs Lymphoma:
General
Leukemia--lymphoid neoplasms w/widespread involvement of BM. Tumor cells found in peripheral blood

Lymphoma--discrete tumor masses arising from LNs

Presentation often blur definitions
Hodgkin's Lymphoma:
Key Finding
Nodal Involvement (Pattern)
Presentation
Associated Virus
Presence of Reed-Sternberg cells = HALLMARK

Lozalized, single group of nodes involved; contiguous spread

B symptoms--fever, night sweats, weight loss

50% cases a/w EBV
Non-Hodgkin's Lymphoma:
Nodal Involvement (Pattern)
Presentation
Associated Virus
May be associated with HIV, immunosuppression

Multiple, peripheral nodes; extranodal involvement common, NONCONTIGUOUS SPREAD

Peak incidence 20-40
Nodular sclerosing lymphoma:
Hodgkin's/Non-Hodgnkin's
Prognosis
Most common HODGKIN'S lymphoma; excellent prognosis bc has high lymphocyte and low R-S cells
Burkitt's lymphoma--starry sky appearance from macrophage ingestion of tumor cells
Hodgkin's Disease (Reed-Sternberg cells)--binucleate RS cells displaying prominent inclusion-like nucleoli surrounded by lymphocytes and other reacting inflammatory cells. RS cell is necessary but insufficient pathologic finding for diagnosis of Hodgkin's disease.
Acute lymphocytic leukemia--affects children less than 10 years of age
Acute myelocytic leukemia with Auer rods (long arrow); affects adolescents to young adults, but most commonly diagnosed in older adults.
Chronic lymphocytic leukemia--lymphocytes are excessively fragile and are easily destroyed during slide preparation, forming "smudge cells". Affects individuals older than 60 years of age.
Chronic myeloid leukemia: promyelocytes and myelocytes seen adacent to a vascular structure. Affects individuals from 30 to 60 years of age.
Burkitt's lymphoma;
Genetics
Histologic appearance
Virus association
t(8;14) c-myc gene

Starry sky appearance

Associated with EBV
t(11;14)
Mantle cell lymphoma
CD5+ Lymphoma
Mantle cell lymphoma
t(14;18)
Follicular lymphoma
bcl-2 expression
Follicular lymphoma
Acute vs Chronic Leukemia:
General
Acute:
Rapid onset, rapidly progressive
Over 50% myeloblasts (AML) or lymphoblasts (ALL) in BM
Numerous blast cells in smear (>20%)
Often a/w pancytopenia

Chronic leukemia:
Insidious onset, gradual progression
Mature cells (<5% blasts)
Can be either CML or CLL
A/w HSM and LAD
ALL:
Characteristics
Bone pain common
Good prognosis in children
PAS(+) (periodic acid-schiff stain)
AML:
Characteristics
AUER RODS
CD13/33+
PAS(-)
Median age of onset is 50
CLL:
Characteristics
Adults over 50
B cell markers (CD19/20?)
SMUDGE CELLS
CML:
Characteristics
80% progress to AML
Adults 25-60
PHILADELPHIA CHROMOSOME (t9;22) ALWAYS PRESENT
Multiple Myeloma:
Pathophys
Presentation
Monoclonal plasma cell cancer (fried egg appearance) that arises in marrow and produces large amounts of IgG (55%) or IgA

CRAB presentation:
hyperCa2+
Renal insuff
Anemia
Bone lytic lesions/Back pain
Why does multiple myeloma exhibit a protein spike?
Multiple Myeloma results in overproduction of a single antibody form a plasma cell; so will see an M protein spike when graphing out subunits of Ig's (gamma subunit)
Rouleaux formation:
What is it?
a/w?
Stacked poker chip formation of RBCs
Seen in multiple myeloma
Multiple Myeloma--classic bone lytic lesions
Multiple myeloma--abundance of plasma cells. RBCs will often be seen in rouleaux formation, stacked like poker chips.
Why does multiple myeloma cause hypercalcemia?
Make cytokines that build oblasts, stimulate oclasts.
What is a leukemoid reaction?
Often confused w/leukemia; inc'd WBC count with left shift (e.g., 80% bands) and inc'd leukocyte alk phos, usually due to infection.
Which chronic myeloproliferative disorder is Philadelphia chromosome positive?
CML
Which chronic myeloproliferative disorder is JAK2 mutation positive?
Polycythemia vera
Essential thrombocytosis
Myelofibrosis

(CML is only negative)
Polycythemia vera:
What is it?
Presentation
Abnl clone of hematopoietic stem cells increasingly sensitive to growth factors

Erythrocytosis despite low EPO

Results in HA, HSM, hyperviscosity
Essential thrombocytosis:
What is it?
Production of PLT despite low thrombopoietin levels
Myelofibrosis:
What is it?
Fibrotic obliteration of bone marrow

See tear drop cells (bone marrow is crying because it's fibrosed :( )
Teardrop cells
Myelofibrosis
What is the role of JAK2?
Plays role in hematopoietic GF signaling
Most common leukemia in children
"ALL"
Most common leukemia in adults in US
"CLL"
Most common lymphoma in US
"Diffuse large B-cell lymphoma"
Reed-Sternberg cells
"Hodgkin's lymphoma"
AML a/w Down syndrome
"M7AML"
Leukemia with more mature cells and less than 5% blasts
"Chronic leukemia"
AML that are CD13 and CD33 (+)
"M0-M6AML"
Particularly a/w EBV
"Burkitt's and Hodgkin's Lymphomas"
Characteristic Auer rods
"AML (particularly M2 and M3)"
A/w long term celiac disease
"Intestinal T Cell Lymphoma"
Greater than 20% blasts in marrow
"Acute leukemia"
Myelodysplastic syndromes have a tendency to progress to ________
"AML"
Myeloproliferative disorders may progress to _________
AML
AML that is CD 41 and CD 61 (+)
"M7AML"
PAS(+) acute leukemia
"ALL"
Commonly presents with bone pain
ALL
Viscous blood, headache, plethora, splenomegaly, and low erythropoietin
"Polycythemia vera"
Leukemia equivalent of Burkett's lymphoma
"L3 or B variant ALL"
Lymphoma equivalent of CLL
Small lymphocytic lymphoma
Numerous basophils, splenomegaly, and negative for leukocyte alkaline phosphatase (LAP)
"CML"
Most common neonatal leukemia
M7AML
Always positive for the Philadelphia chromosome (t9;22)
CML
Only AML that is CD 13 and CD 33 (-)
M7 AML
"Starry-sky pattern" due to phagocytosis of apoptotic tumor cells
Burkitt's lymphoma
Always associated with the BCR-ABL genes
CML
A/w Sjogren syndrome, Hashimoto's thyroiditis, and H. pylori
Marginal cell MALToma
Acute leukemia positive for peroxidase
AML
Solid sheets of lymphoblasts in marrow
ALL
PAS(-) acute leukemia
AML
Compare the age distribution of those affected by Hodgkin's lymphoma to those affected by non-Hodgkin's lymphoma.
HL: bimodal, young and old

NHL: 20-40 (fills gap)
What is the most common type of NHL in adults?

In children?
Adults: Diffuse large B cell lymphoma

Children: Lymphoblastic lymphoma
What lab findings are indicative of disseminated intravascular coagulation?
Elevated PT/INR
Elevated PTT
Eleveated BT
Dec'd PLT
Elevated D-dimer
What is the structure of HbH?

What disease results in its production?
4 beta subunits

alphta-thal
What is the structure of Hb Barts?

What disease results in its production?
4 gamma subunits

hydrops fetalis
Hematologic disease:
(+)Ham's test
Paroxysmal nocturnal hemoglobinuria
Hematologic disease:
Heinz bodies
G5PD def
Hematologic disease:
Basophilic stippling
Pb poisoning
Hematologic disease:
(+)osmotic fragility test
Hereditary spherocytosis
Hematologic disease:
(+)DEB test
Fanconi's anemia
Hematologic disease:
D-dimers
DIC
Hematologic disease:
Coomb's (+)
Used to ID autoimmune hemolytic anemia
Hematologic disease:
Coomb's (-)
Used to ID non-autoimmune hemolytic anemia
Hematologic disease:
( +) ristocetin test
vW Dz
What are the causes of aplastic anemia?
Radiation, Benzene, EBV, Fanconi's anemia, etc.
A patient develops hypercalcemia from the most common primary tumor arising within bones in adults.

What lab findings you expect in this patient?
Multiple myeloma

Expect:
monoclonal Ab spike
Punched out lytic bone lesions
Bence-Jones proteins in urine
Rouleaux formation on blood smear
What findings are a/w hereditary spherocytosis?
Poz osmotic fragility test
Coombs' neg
Pigmented gallstones
Splenomegaly
Anemia
Jaundice
What lab findings allow you to distinguish iron deficiency anemia from a microcytic, hypochromic anemia resulting from thalassemia?
Fe def:
Dec'd Serum Fe
Inc'd TIBC
Dec'd Ferritin
NO TARGET CELLS

Thal:
Nl Fe
Nl TIBC
Nl Ferritin
TARGET CELLS PRESENT
A child anemic since birth now has been cured with splenectomy.

What is the disease?
Hereditary spherocytosis
A patient with anemia, hypercalcemia, and bone pain receives a bone marrow biopsy which reveals plasma cells (large, round, off-center nucleus).

What is the diagnosis and what would you expect to see on urinalysis?
Multiple myeloma

Won't see anything on U/A, need UPEP (electrophoresis for your pee) to see Bence-Jones Proteins
What neoplasms are a/w AIDS?
Kaposi's
Invasive squamous cell cancer
CNS
lymphoma
NHL
A heart failure patient is newly diagnosed with cancer and is being evaluated for chemotherapy.
Which chemotherapeutic agent should be avoided in this pt?
Doxorubicin or Daunorubicin--cardiotoxicity!
Chromosomal analysis of a leukemia patient reveals the presence of the Philadelphia chromosome t(9;22).

What is the treatment?
Imatinib
After a normal spontaneous vaginal delivery, the new mom bleeds profusely from her vagina and later from her gums.

What abnormal lab values would you suspect?
DIC probably to amniotic fluid embolism!

Inc'd BT
Inc'd PT, PTT
Dec'd PLT
Inc'd D-Dimer
A 11 year-old child presents with a chronic non healing ulcer on his foot and imaging shows a small calcified spleen.
What drug can improve his symptoms?
This is sickle cell; tx w/hydroxyurea to create HgF to better carry O2 to tissues
NSAIDs inhibit the production of which substance important in platelet aggregation?
Thromboxane
Does HbF have more or less affinity for 2,3-bisphosphoglycerate?
Less affinity
MOA:
Streptokinase
Converts plasminogen to plasmin
MOA:
ASA
Irreversible COX-1 inhibitor
MOA:
Clopidogrel
blocks ADP receptors
MOA:
Abciximab
Binds GpIIb/IIIa
MOA:
Tirofiban
Binds GpIIb/IIIa
MOA:
Ticlopidine
Blocks ADP receptors
MOA:
Enoxaparin
Catalyzes Antithrombin III formation and activation
MOA:
Eptifibatide
Binds GpIIb/IIIa