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Step1 > Hematology and Oncology > Flashcards

Hematology and Oncology Flashcards

1
Q

Anisocytosis

A

varying sizes

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2
Q

Poikilocytosis

A

varying shapes

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3
Q

Polychromasia

A

bluish color on wright giemsa stain of reticulocytes represent residual ribosomal RNA

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4
Q

Erythrocytes life span? energy source? membrane contains what transporter?

A

120 days
Glucose
Cl-/HCO3 antiporter to expcort HCO3 and transport CO2

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5
Q

Thrombocyte life span? derived from? contains? stored?

A

8-10 days
Megakaryocytes (proliferation due to thrombopoietin)
Contains dense granules (ADP and Ca) and alpha granules (vWF,fibrinogen,fibronectin)
Approximately 1/3 of platelet pool is stored in the spleen

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6
Q

Thrombocytopenia

A

decreased platelet function results in petechiae

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7
Q

receptor for vWF? fibrinogen?

A

vWF is GpIIb

fibrinogen is GPIIb/IIIa

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8
Q

Leukocytes

A

two types :

1) granulocytes (neutrophils (60%), eosinophils (3%), basophils (1%), mast cells)
2) mononuclear cells ( monocytes (6%), lymphocytes (30%))

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9
Q

Neutrophils

A

acute inflammatory response cells

increases in bacterial infections

phagocytic

multilobed (hypersegmented have 6+ lobes) and are seen in B12/B9(folate) deficiency

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10
Q

Neutrophils contain

A 
Granules:
leukocyte alkaline phosphatase (LAP)
collagenase
lysozyme
lactoferrin
Azurophilic granules(lysosomes):
proteinases
acid phosphatase
myeloperoxidase
beta-glucuronidase
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11
Q

Increased band cells (immature neutrophils)

A

reflect states of increased myeloid proliferation (bacterial infections, CML)

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12
Q

Important neutrophil chemotactic agents

A 
C5a
IL8
LTB4
Kallikrein
platelet activating factor
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13
Q

Monocytes

A

macrophage (tissues) but monocytes (blood)

have a large kidney shaped nucleus and frosted glass cytoplasm

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14
Q

Macrophages

A

phagocytose bacteria, cellular debris, and senescent RBCs

activated by gamma interferon and can function as APC via MHC II

have different name in different tissues

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15
Q

Septic shock is due to what binding

A

Lipid A from bacterial LPS binds CD14 on macrophages to initiate septic shock

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16
Q

Eosinophils defend against? produce? lobed nucleus?

A
  • Defend against helminthic infections
  • Bilobate nucleus
  • Produce histaminase, major basic protein (MBP, a helminthotoxin), eosinophil peroxidase, eosinophil cationic protein, and eosinophil derived neurotoxin
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17
Q

Causes of eosinophilia

A 
parasites
Asthma
Churg Strauss syndrome (allergic granulomatosis)
Chronic adrenal insufficiency
Myeloproliferative disorders
Allergic processes
Neoplasia
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18
Q

Basophils

A

Mediate allergic reactions

basophilic granules that contain heparin (anticoagulant) and histamine (vasodilator)

Leukotrienes synthesized and released on demand

basic stains

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19
Q

Basophilia

A

uncommon

sign of myeloproliferative disease particularly CML

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20
Q

Mast cells

A

mediate allergic reactions in local tissues (type 1 hypersensitivity)

basophillic granules

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21
Q

Activation of mast cells

A

bind Fc portion of IgE to membrane

activated by tissue trauma, c3a and c5a, surface IgE crosslinking by antigen (IgE receptor aggregation) –> degranulation –> release of histamine, heparin, tryptase, and eosinophil chemotactic factors

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22
Q

Dendritic cells

A

phagocytic antigen presenting cells

link innate and adaptive immune systems

express MHC class II and Fc receptors on surface

called langerhans cells in the skin

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23
Q

Lymphocytes

A

refer to B cells, T cells, and NK cells

B and T cells mediate adaptive immunity
NK cells are part of the innate immune response

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24
Q

B cells immune response and development

A

Humoral immune response

from stem cells in bone marrow and matures in marrow

migrate to peripheral lymphoid tissue (lymph nodes, white pulp of spleen, unencapsulated lymphoid tissue)

when antigen is encountered, B cells differentiate into plasma cells (which produce antibodies) and memory cells

APC functioning too

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25
Q

T cells immune response and development

A

Mediate cellular immune response

From stem cells in the bone marrow but mature in the thymus

Differentiate into cytotoxic T cells (express CD 8, recognize MHC I) , helpher T cells (express CD 4, recognize MHC II), and regulatory T cells

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26
Q

Costimulatory signal necessary for T cell activation

A

CD28

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27
Q

Most circulatory lymphocytes are

A

T cells (80%)

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28
Q

Plasma cells

A

Produce large amounts of antibody specific to a particular antigen

“clock face” chromatin distribution and eccentric nucleus

abundant RER and well developed Golgi apparatus

found in bone marrow and normally do not circulate in peripheral blood

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29
Q

multiple myeloma

A

plasma cell cancer that accumulates in bones and weakens them

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30
Q

Fetal erythropoiesis occurs in? time frame?

A

yolk sac for 3-8 wks
Liver for 6 weeks to birth
Spleen 10-28 weeks
Bone marrow 18 weeks to adult

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31
Q

Embryonic globins?

A

zeta (ζ) and epsilon (ɛ)

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32
Q

Fetal hemoglobin

A

HbF=α2γ2

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33
Q

Adult hemoglobin

A

HbA1=α2β2

HbA2 (small amounts)=α2δ2

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34
Q

fetal hemoglobin –> adult

A

Alpha always
Gamma goes
Becomes beta

HbF=α2γ2
HbA1=α2β2

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35
Q

Fetal hemoglobin

A

HbF has higher affinity for O2 due to less avid binding of 2,3-BPG, allowing HbF to extract O2 from maternal hemoglobin (HbA1 and HbA2) across the placenta

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36
Q

Blood groups: Group A

A

type A has antigen A on surface

antibodies in plasma are Anti-B (IgM)

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37
Q

Blood groups: Group B

A

type B has antigen B on surface

anitbodies in plasma are Anti-A (IgM)

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38
Q

Blood groups: group AB

A

type AB has antigens to A and B on surface

no antibodies

universal recipient of RBC
unviersal donor of plasma

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39
Q

Blood groups: group O

A

Type O has no antigens on surface

Antibodies against A and B (IgM and IgG) in plasma

Universal donor of RBCs
Universal recipient of plasma

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40
Q

Rh classification: Rh + RBC

A

antigen on surface is Rh(D)
No antibodies in plasma

can receive Rh+ or Rh- blood

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41
Q

Rh classification: Rh - RBC

A

no antigen on surface

Anti-D (IgG) antibodies in plasma

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42
Q

Rh hemolytic disease of the newborn (erythroblastosis fetalis)

A

Mom Rh- but fetus Rh+

after first pregnancy mother will form maternal anti-D IgG

Subsequent pregnancy have anti-D igG crosses the placenta causing HDN in fetus (jaundice, kernicterus, hydrops fetalis)

Prevent: treat mother with anti-D Ig during and after each pregnancy to prevent anti-D IgG formation

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43
Q

ABO hemolytic disease of newborn (erythroblastosis fetalis)

A

Type O mothers with type A or B fetus

preexisting maternal anti-A or anti-B IgG antibodies cross placenta –> HDN in the fetus

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44
Q

Hemoglobin electrophoresis (movement from negative cathode to positive anode)

A

HbA (most negative due to glutamic acid) > HbF > HbS>HbC

missence mutations in HbS and HbC replace glutamic acid (-) with valine (neutral) and lysine (+) –> impacts net protein charge

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45
Q

Plasmin pathway

A

Plasminogen becomes plasmin via tPA

plasmin degrades many blood plasma proteins, including fibrin clots. The degradation of fibrin is termed fibrinolysis (cleavage of the fibrin mesh and destruction of coagulation factors)

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46
Q

Kinin cascade

A

HMWK becomes bradykinin via kallikrein

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47
Q

What does bradykinin do

A

increase vasodilation, permeability, pain

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48
Q

Extrinsic pathway

A

external trauma that causes blood to escape from the vascular system

favtor VII –> VIIa

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49
Q

Intrinsic pathway

A

activated by trauma inside the vascular system, and is activated by platelets, exposed endothelium, chemicals, or collagen.

This pathway is slower than the extrinsic pathway, but more important. It involves factors XII, XI, IX, VIII.

XII –> XIIa which is then used to make XI–>XIa etc

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50
Q

Combined pathway

A

Both pathways meet and finish the pathway of clot production in what is known as the common pathway. The common pathway involves factors I, II, V, and X.

note: II is prothrombin which becomes thrombin or IIa
I is fibrinogen which becomes Ia fibrin monomers

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51
Q

Vitamin Ks relationship to the coagulation cascade

A

oxidized vitamin K becomes reduced vitamin K via epoxide reductase

reduced vitamin k acts as a cofactor that activates factors II, VII, IX,X,C,S

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52
Q

Where does warfarin act and how do you reverse its effects

A

warfarin inhibits epoxide reductase and prevents oxidized vitamin k from becoming reduced vitamin k

You can reverse its effects with vitamin k admin, but FFP and PCC are immediate reversal

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53
Q

Neonates and vitamin k

A

neonates dont have enteric bacteria which produce vitamin k

early administration of vitamin k overcomes neonatal deficiency/coagulopathy

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54
Q

Which factor has the longest half life? which has the shortest?

A

factor VII is shortest

Factor II is longest

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55
Q

vWF carriers/protescts factor ____

A

factor VIII of intrinsic pathway

they together enter the combined pathway where they join factor VII of extrinsic to form Xa of the combined pathway

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56
Q

Anticoagulation with protein C

A

protein C is activated with thrombin-thrombomodulin complex (endothelial cells)

it then acts with protein S to cleave and inactivate Va (combined pathway) and VIIIa (intrinsic pathway)

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57
Q

Antithrombin

A

inhibits activated forms of factors II,VII,IX,X,XI,XII but the principal targets are thrombin (IIa) and factor Xa

activity is enhanced by heparin

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58
Q

Factor V leiden mutation

A

produces a factor V resistant to inhibition by activated protein C

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59
Q

Primary hemostasis steps

A

1) Injury - endothelial damage causes transient vasoconstriction via neural stimulation reflex and endothelin released from damage cell
2) Exposure- vWF (from weibel palade bodies of endothelial cells and alpha granules of platelets) binds exposed collagen
3) Adhesion - platelets bind vWF via GpIb and undergo conformaitional change. They release ADP(helps platelets adhere) and Ca, TxA2.
4) ADP binds P2Y12 receptor and induces GpIIb/IIIa expression on platelet surface
5) aggregation- fibrinogen binds GpIIb/IIIa receptors and links platelets
6) temporary plug –> secondary coagulation cascade

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60
Q

where does vWF get released from

A

vWF (from weibel palade bodies and alpha granules of platelets)

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61
Q

Pro aggregation factors?

A

TXA2
Decreased blood flow
Increased platelet aggregation

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62
Q

Anti-aggregation factors?

A

PGI2 and NO released by endothelial cells
Increased blood flow
Decreased platelet aggregation

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63
Q

Thrombogenesis

A

the formation of insoluble fibrin mesh

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64
Q

Aspirin irreversibly binds ____ thereby inhibiting ____ synthesis

A

cyclooxygenase

TXA2 synthesis from arachidonic acid

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65
Q

Pt presents with low iron, high TIBC, low ferritin, high free erythrocyte protoporphyrin, increased RDW

A

Iron deficiency anemia

microcytosis and hypochromasia (increased central pallor)

symptoms: conjunctival pallor and spoon nails (koilonychia), glossitis, cheilosis, plummer vinson syndrome (triad of iron deficiency anemia, esophageal webs, and dysphagia)

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66
Q

α-thalassemia

A

α globin gene deletions which causes decreased α globin synthesis

can be cis (same crhomosome) or trans. But Cis is worse

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67
Q

Types of α thalassemia

A

α thalassemia minima (lose only 1) - no anemia, silent carrier

α thalassemia minor (lose 2) - mild microcytic, hypochromic anemia

Hemoglobin H disease (HbH, lose 3) - excess β forms β4. Moderate to severe microcytic hypochromic anemia

Hemoglobin Barts disease (Hb Barts, lose 4) - excess γ forms γ4 which is hydrops fetalis

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68
Q

β thalassemia

A

Point mutations in splice sites and promoter sequences causes a decrease in β-globin synthesis

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69
Q

β thalassemia types

A

β thalassemia minor (heterozygote)- β chain is underproduced. asymptomatic. Increase in HbA2 on electrophoresis

β thalassemia major (homozygous) - β chain is absent causing severe microcytic hypochromic anemia with target cells and increase anisopoikilocytosis requiring blood transfusion. Results in increased HbF and HbA2

HbS/ β thalassemia heterozygote: mild to moderate sickle cell disease depending on amount of β globin production

70
Q

Patient presents with a crew cut appearance on skull xray due to marrow expansion. He also has what is described as a “chipmunk facies”

A

β thalassemia major

pts also has extramedullary hematopoiesis –> hepatosplenomegaly

Also at increased risk of aplastic crisis due to parvovirus B19

HbF is protective in the infant and disease becomes symptomatic only after 6 months when fetal hemoglobin declines

71
Q

Lead poisoning

A

lead inhibits ferrochelatase and ALA dehydratase which decreases heme synthesis and increases RBC protoporphyrin. Also inhibits rRNA degradation which results in basophilic stippling in RBCs

72
Q

Symptoms of lead poisoning

A

L ead lines on gingivae (burton lines) and on metaphyses of long bones on xray
E ncephalopathy and erythrocyte basophilic stippling
A bdominal colic and sideroblastic anemia
D rops-wrist and foot drop

73
Q

Sideroblastic anemia

A

form of anemia in which the bone marrow produces ringed sideroblasts rather than healthy red blood cells (erythrocytes).

genetic, acquired, or reversible forms

most common reversible form is due to alcohol

74
Q

Pt presents with the following labs: increased iron, low TIBC, increased ferritin. There are ringed sideroblasts with iron-laden prussian blue stained mitochondria seen in bone marrow. Peripheral blood smear has basophilic stippling of RBCs

A

Sideroblastic anemia

tx: pyridoxine (B6, cofactor for ALA synthase)

75
Q

Pt presents with RBC macrocytosis, hypersegmented neutrophils, glossitis

A

Megaloblastic anemia

impaired DNA synthesis causes maturation of nucleus of precursor cells in bone marrow delayed relative to maturation of cytoplasm

76
Q

Pt presents with increased homocysteine, normal methylmalonic acid. No neurologic symptoms

A

Folate deficiency (i.e. due to alcohol, pregnancy, etc)

77
Q

Pt presents with increased homocysteine and increased methylmalonic acid. With neurologic symptoms

A

Vitamin B12 (cobalamin) deficiency

dx with the schilling test (dietary insufficiency vs malabsorption)

anemia due to insufficiency may take several years to develop due to livers ability to store B12 (as opposed to folate deficiency)

78
Q

Child presents with failure to thrive, developmental delay, and megaloblastic anemia refractory to folare and B12. Pt has orotic acid in urine. No hyperammonemia. What enzyme is defective?

A

Orotic aciduria due to an inability to convert orotic acid to UMP (de novo pyrimidine synthesis pathway) because of a defect in UMP synthase.

tx with uridine monophosphate or uridine triacetate to bypass mutated enzyme

79
Q

Nonmegaloblastic anemia

A

macrocytic anemia in which DNA synthesis is unimpaired. RBC macrocytosis without hypersegmented neutrophils

caused by alcoholism and liver disease

80
Q

Pt presents with increased HbF, short stature, craniofacial abnormalities, and UE malformations (triphalangeal thumbs) in up to 50% of cases

A

Diamond-blackfan anemia - form of nonmegaloblastic anemia

Rapid onset anemia within first year of life due to instrinsic defect in erythroid progenitor cells

81
Q

Pt presents with low haptoglobin, increased schistocytes on blood smear, hemoglobinuria, hemosiderinuria, and urobilinogen in urine. There is also an increase in unconjugated bilirubin

A

Intravascular hemolysis (normocytic, normochromic anemia)

notable causes are mechanical hemolysis, paroxysmal nocturnal hemoglobinuria, microangiopathic hemolytic anemias

82
Q

Pt presents with macrophages in spleen clearing RBCs. Spherocytes in peripheral smear. No hemoglobinuria/hemosiderinuria. CAn also present with urobilinogen in urine

A

extravascular hemolysis (normocytic, normochromic anemia)

83
Q

Pt has SLE and now has low iron, low TIBC, increased ferritin. Pt was initially normocytic but is not microcytic anemia.

A

Anemia of chronic disease ( nonhemolytic, normocytic anemia)

Inflammation causes an increase in hepcidin release from the liver. Hepcidin binds ferroportin on intestinal mucosal cells and macrophages thus inhibiting iron transport

decrease release of iron from macrophages and decrease iron absorption from gut

84
Q

Pt presents with fatigue, malaise, pallor, purpura, mucosal bleeding, petechiae, infection, a drop in reticulocyte count, and increase in EPO (erythropoietin stimulates RBC production)

A

Aplastic anemia- Body stops producing enough new blood cells due to failure or destruction of myeloid stem cells

Pancytopenia characterized by anemia, leukopenia, and thrombcytopenia

Decrease in reticulocyte count with an increase in EPO

85
Q

Extravascular hemolysis due to defect in proteins interacting with RBC membrane skeleton and plasma membrane. Resulting in small round RBCs with less surface area and no central pallor –> premature removal by spleen causes an increase in MCHC

A

Hereditary spherocytosis

splenomegaly, aplastic crisis (parvovirus B19 infection)

labs find increased fragility in osmotic fragility test which causes normal to decreased MCV with abundance of cells.

tx with splenectomy

86
Q

G6PD deficiency causes ______ hemolysis and reduced ____ which increases RBC susceptiility to _______ after eating fava beans. Pt presents with back pain and hemoglobinuria after eating fava beans. Blood smear shows RBCs with _____ bodied and _____ cells.

A 
extravascular and intravascular hemolysis
glutathione
oxidant stress
Heinz bodies (Hb precipitation)
Bite cells
87
Q

This deficiency causes hemolytic anemia in newborns. It causes a drop in ATP which results in the formation of rigid RBC –> _______ hemolysis. There is an increase level of _______ and thus a drop in hemoglobin affinity for O2

A

Pyruvate kinase deficiency

88
Q

Paroxysmal nocturnal hemoglobinuria

A

increase complement mediated intravascular RBC lysis

associated with aplastic anemia

triad of Coombs negative hemolytic anemia, pancytopenia, venous thrombosis.

Pts present with red or pink urine due to hemoglobinuria

CD55/59 - RBCs

tx: eculizumab

89
Q

Mutation in paroxysmal nocturnal hemoglobinuria

A

acquired mutation in PIGA gene causing impaired synthesis of GPI anchor for decay accelerating factor [DAF/CD55] and membrane inhibitor of reactive lysis [MIRL/CD59] that protects RBC membrane from complement

increases incidence of acute leukemias

90
Q

HbC disease

A

glutamic acid to lyCine (lysine) mutation in beta globin

causes extravascular hemolysis

pt with HbSC is milder than HbSS

hemoglobin crystals inside RBCs, target cells

91
Q

Sickle cell anemia

A

HbS point mutation causes beta chain AA glutamic acid to become valine

extra and intravascular hemolysis

Like in beta thalassemia, pt can present with a crew cut on skull xray due to marrow expansion from increased erythropoiesis

Pts can also present with dactylitis or painful swelling of hands/feet

sickling in renal medulla (drop in PO2) –> renal papillary necrosis –> microhematuria

tx with hydroxyurea (increases HbF) and hydration

92
Q

In extravascular hemolysis RBCs are phagocytized by macrophages in the _________

A

in the spleen and liver.

93
Q

In intravascular hemolysis RBCs are due to physical trauma or complement fixation in the _________

A

in the blood vessels

94
Q

Warm Autoimmune hemolytic anemia

A

IgG

chronic anemia seen in SLE and CLL and with certain drugs

“WARM weather is Great

95
Q

Cold Autoimmune hemolytic anemia

A

IgM

Acute anemia triggered by cold; seen in CLL, Mycoplasma pneumo, Mono.

“COLD weather is MMMiserable”

96
Q

Autoimmune hemolytic anemia

A

Cold and warm type

RBC agglutinated may cause painful blue fingers and toes with cold exposure

Coombs +

97
Q

Anti-Ig antibody added to patients RBC. RBC agglutinate if coated with Ig

A

Direct coombs test

98
Q

Normal RBCs added to patients serum. If serum has anti RBC surface Ig, the RBCs agglutinate when coombs reagent added

A

Indirect coombs test

99
Q

Microangiopathic anemia

A

RBCs are damaged when passing through obstructed or narrowed vessel lumina

forms schistocytes due to mechanical destruction and formation of fragmented RBCS

100
Q

Macroangiopathic anemia

A

Prosthetic heart valves and aortic stenosis may also cause hemolytic anemia secondary to mechanical destruction of RBCs

schistocyte formation

101
Q

Iron overload

A

hemochromatosis

increased serum iron, drop in transferrin or TIBC (TIBC indirectly measures transferrin), increase ferritin (storage), and large increase in percent transferrin saturation

102
Q

Leukopenia
Neutropenia
Lymphopenia
Eosinopenia

A

Leukopenia is a general decrease in WBCs (leukocytes) found in blood which increases risk of infection

Neutropenia (<1500, if <500 then severe infections result)

Lymphopenia (<1500, <3000 in children)

Eosinopenia (<30)

corticosteroids cause neutrophilia, despite causing eosinopenia and lymphopenia

103
Q

Left shift

A

defines as a shift towards more immature cells in the maturation process

i.e. in infection or inflammation where we get neutrophilia where there is an increase in neutrophil precursors, such as band cells and metamyelocytes, in peripheral blood

104
Q

Leukoerythroblastic reaction

A

when a left shift is seen with immature RBCs

occurs with severe anemia (physiologic response) or marrow response

105
Q

Porphyrias

A

Defective heme synthesis that lead to accumulation of heme precursors

lead can inhibit specific enzymes needed in heme synthesis leading to similar condition

106
Q

What enzyme is affected in lead poisoning and what substrate accumulates

A
  • Ferrochelatase and ALA dehydratase

- Protoporphyrin, ALA in blood

107
Q

What enzyme is affected in acute intermittent porphyria and what substrate accumulates

A
  • Porphobilinogen deaminase

- Porphobilinogen, ALA

108
Q

What enzyme is affected in porphyria cutanea tarda and what substrate accumulates

A
  • Uroporphyrinogen decarboxylase

- Uroporphyrin –> tea colored urine

109
Q

Pt presents with microcytic anemia, basophilic stippling in peripheral smear, ringed sideroblasts in bone marrow. Most notable physical symptoms are a headache. memory loss, and demyelination. The pt child also presents with mental deterioration

A

Lead poisoning

110
Q

Pt presents with painful abdomen, port wine colored urine, polyneuropathy, psychological disturbances. All of which is precipitated by drugs, alcohol, and starvation

A

Acute intermittent porphyria

111
Q

Pt was dx with Hep c and now presents with blistering cutaneous photosensitivity and hyperpigmentation. It is exacerbated with alcohol consumption.

A

Porphyria cutanea tarda

112
Q

Pt is a child that presents with nausea, vomiting, gastric bleeding, lethargy, and scarring that has now lead to a GI obstruction. Child mentions eating something that looked like “Candy”

A

Iron poisoning

cell death due to peroxidation of membrane lipids

tx with chelation

113
Q

PT

A

common and extrinsic pathway (I,II,V,VII,X)

Play Tennis outside

114
Q

PTT

A

Play Table Tennis Inside

common and intrinsic pathway

115
Q

INR (internationalized normalized ratio)

A

1= normal
>1 is prolonged

based on PT for following patients on warfarin

116
Q

mixing study

A

normal plasma is added to patients plasma

clotting factor deficiencies should correct the PT or PTT

Factor inhibitors will not be corrected

117
Q

Hemophilia? A?B?C?

A

Hemophilia is the inability to clot blood due to an intrinsic pathway coagulation defect (PTT)

A? factor VIII (A is eight)
B? factor IX ( Bnine)
C? factor XI (Celeven)

tx is desmopressin + whatever factor is missing

118
Q

Hemarthroses

A

bleeding into joints due to hemophilia

119
Q

Vitamin K deficiency

A

Bleeding time normal but see both an increase in PT (extrinsic) and PTT (intrinsic)

120
Q

Platelet disorders

A

Defects in platelet plug formation causes an increase in bleeding time and possibly platelet count

121
Q

Bernard soulier syndrome

A

Drop in platelet count and rise in bleeding time

Defect in platelet plug formation due to a decrease in GpIb which prevents adhesion to vWF

Large platelets

Does not correct with mixing studies

122
Q

Glanzmann thrombasthenia

A

Unchanged platelet count and increased bleeding time

Defect in GpIIb/IIIa causing a problem with platelet to platelet aggregation

blood smear shows no platelet clumping

123
Q

Hemolytic uremic syndrome

A

Decreased platelet count and increased bleeding time

thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure

In children: diarrhea and EHEC infection
In adults: no diarrhea and does not need EHEC

124
Q

Immune thrombocytopenia

A

Decrease platelet count and increased bleeding time

Anti-GpIIb/IIIa antibodies cause splenic macrophages to consume the platelet antibody complex

labs show an increase in megakaryocytes on bone marrow biopsy

125
Q

Thrombotic thrombocytopenic purpura

A

Decrease platelet count and increased bleeding time

Inhibition or deficiency of ADAMTS13 (vWF metalloprotease) which results in degradation of vWF multimers. This causes increase of multimers and increases platelet adhesion and aggregation –> thrombosis

126
Q

Pentad of sx for thrombotic thrombocytopenic purpura

A 
Fever
Microangiopathic hemolytic anemia
Thrombocytopenia (low platelet count)
Renal failure
Neurologic symptoms

can also see schistocytes

127
Q

Von willebrand disease

A

Increase in bleeding time and increase in PTT (instrinsic)

vWF acts to carry and protect factor VIII

most common inhereted bleeding disorder

tx with desmopressin which releases the vWF thats stored in endothelium

128
Q

Disseminated intravascular coagulation

A

DIC: drop in platelet count, Increase bleeding time, increase PT and PTT

widespread activation of clotting which causes a deficiency in clotting factors resulting in a bleeding state

can see schistocytes, increased fibrin degradation products (D-dimers), decrease fibrinogen, decrease factors V and VIII

129
Q

Pt presents with a decrease in the inhibition of factors IIa and Xa

A

IIa is thrombin

Antithrombin deficiency

main effect seen is that it diminishes the increase in PTT seen with heparin admin

130
Q

In factor V leiden, there is a mutant factor V due to ___ to ____ point mutation resulting in ______ mutation near the cleavage site

A

G–> A DNA point mutation

Arg506Gln mutation near cleavage site

resistant to degradtion by activated protein C

DVT, cerebral vein thromboses, recurrent pregnancy loss

131
Q

Pt has a decreased ability to inactivate factors Va and VIIIa With an increased risk of thrombotic skin necrosis with hemorrhage after administration of warfarin

A

Protein C or S deficiency

C cancels and S stops coagulation

132
Q

Prothrombin gene mutation

A

mutation in the 3’ untranslated region –> increased production of prothrombin (II)

this caueses an increase in factor II plasma levels and ultimately clots

133
Q

Fresh Frozen Plasma/ Prothrombin complex concentrate

A

FFP - Increase coagulation factors and plasma protein

PCC- factors II, VII, IX, X, C, S

134
Q

Cryoprecipitate

A

contains fibrinogen

factors VIII
factor XIII
vWF
Fibronectin

135
Q

Pt presents with low grade fever, night sweats, weight loss. On biopsy you find that localized single group of nodes are affected. Contiguous spread is noted. You also noticed Reed-sternberg cells on histology.

A

Hodgkin lymphoma (better than non-Hodgkins)

young adulthood or >55 years old

Men mostly

EBV associated

Reed-sternberg cells are CD15+ and CD30 + B cell origin

136
Q

Patient presents with a low grade fever, night sweats, weight loss. On biopsy you notice multiple lymph nodes are involved with pathology and that there is extranodal noncontiguous spread

A

Non-hodgkin lymphoma

majority are B cells and a few are T cells

both kids and adults

May be associated with HIV and autoimmune disease

137
Q

Prognosis of different hodgkin lymphoma types

A

Nodular is most common
lymphocyte rich is best prognosis
Mixed cellularity - eosinophilia seen in immunocompromised pt
lymphocyte depleted seen in immunocompromised

138
Q

“Starry sky” appearance, sheets of lymphocytes with interspersed “tingible body” macrophages

A

Burkitts lymphoma

adolescents or young adults “B=Big Kids”

139
Q

This lymphoma has a starry sky appearance on histo and is a common cause of jaw lesions (endemic form) and pelvis or abdomen (sporadic form). What is the genetic mutation for this lymphoma?

A

Burkitss lymphoma

t(8;14) - translocation of c-myc and heavy chain Ig (14)

“BurKIDS=Big kids are 8 and 14. 8 year olds like MYCdonalds but by 14 they are heavy”

140
Q

This is the most common type of non-hodgkins lymphoma in adults with only 20% being in children. What is the genetics behind it

A

Diffuse large B cell lymphoma

alterations in Bcl-2 and Bcl-6

141
Q

Lymphoma that has an indolent course with “waxing and waning” lymphadenopathy in adults. What is the genetics behind it

A

Follicular lymphoma

t(14,18) - translocation of heavy chain Ig (14) and BCL-2 (18)

F=Father was heavy by 14 and was a Big CLown for 2 years by 18 yo”

142
Q

Very aggressive lymphoma in adult males that presents with late stage disease. Genetics?

A

Mantle cell lymphoma

t(11;14) - translocation of cyclin D1 (11) and heavy chain Ig (14), CD 5+

143
Q

Lymphoma that is associated with chronic inflammation in adults. genetics?

A

Marginal zone lymphoma

genetics: t(11,18)

144
Q

This lymphoma is considered an HIV/AIDs defining illness and pathogenesis incolves EBV. It needs to be distinguished from toxoplasmosis because it may have mass lesions in the brain

A

Primary central nervous system lymphoma

145
Q

Pts are adults that are commonly from Japan, west africa, and the caribbean. They present with cutaneous lesions, lytic bone lesions, and hypercalcemia

A

adult T cell lymphoma

caused by HTLV or Human T-cell lymphotropic virus type 1 (associated with IV drug use)

146
Q

Patient presents with plaques and skin patches all over their body. What lymphoma are we worried about

A

Mycosis fungoides ( T cell lymphoma) which may progress to Sezary syndrome ( T cell leukemia)

It is a cutaneous T cell lymphoma characterized by atypical CD4+ cells with cerebriform nuclei and intraepidermal neoplastic cell aggregates (pautrier microabscess)

147
Q

Monocolonal plasma cell cancer that arises in the marrow and produces large amounts of IgG or IgA.

A

Multiple myeloma “fried egg appearance”

Most common bone tumor in >40-50 yo

M spike on serum protein electrophoresis

Bence Jones protein - IgG light chains in urine

Rouleaux formation - RBC stacked like poker chips in blood smear

Punched out ytic bone lesions

Clock face chromatin in plasma cells

primary amyloidosis

Intracytoplasmic inclusions containing immunoglobulin

148
Q

multiple myeloma CRAB mnemonic

A

C-calcemia (hyper)
R-renal involvement
A- anemia
B - bone lytic lesions/back pain

149
Q

________ has no CRAB (calcemia,renal,anemia, bone lytic lesions) findings but may progress to multiple myeloma

A

Monoclonal gammopathy of undetermined significance (MGUS)

monoclonal expansion of plasma cells (bone marrow <10% monoclonal plasma cells)

150
Q

M spike due to IgM, no hypercalcemia, no renal involvement, no anemia, no bone lytic lesions

A

Waldenstrom macroglobulinemia

This is a hyperviscosity syndrome with no CRAB findings

must distinguish from multiple myeloma which has M spike due to IgG (55%) or IgA(25%)

151
Q

Stem cell disorder involving ineffective hematopoiesis that results in defects in cell maturation of nonlymphoid lineages. What anomaly is associated with this

A

myelodysplastic syndrome

risk of becoming AML

Pseudo Pelger Huet anomaly - neutrophils with bilobed “duet” nuclei. Typically seen after chemotherapy

152
Q

Leukemia

A

unregulated growth and differentiationof WBCs in bone marrow –> marrow failure –> anemia ( drop in RBC), infection (drop in mature WBCs), and hemorrhage (drop in platelets)

increased WBC in blood (malignant leukocytes in blood)

153
Q

Acute lymphoblastic leukemia/lymphoma

A

lymphoid neoplasm

mostly in children. Worse prognosis in adults

T cell ALL can present as mediastinal mass

Downs syndrome

Blood smear shows increased lymphoblasts

can spread to CNS and testes

154
Q

_________ leukemia/lymphoma is associated with downs syndrome

A

Acute lymphoblastic leukemia/lymphoma

155
Q

Markers for Acute lymphoblastic leukemia/lymphoma

A

TdT +
CD10+
t(12;21) has better prognosis

156
Q

leukemia/lymphoma that is CD20+,CD23+,CD5+ B cell neoplasm. See smudge cells in peripheral blood smear. An autoimmune hemolytic anemai

A

Chronic lymphocytic leukemia/small lymphocytic lymphoma

Age > 60 years (most common adult leukemia)

CLL= crushed little lymphocytes = smudge cells

157
Q

CLL/SLL can under go a _____ transformation into ______

A

richter transformation

diffuse large B-cell lymphoma (DLBCL)

158
Q

Mature B cell tumor in adult males. Cells have filamentous hair like projections

A

Hairy cell leukemia

causes marrow fibrosis –> dry tap on aspiration

massive splenomegaly and pancytopenia

159
Q

Hair leukemia requires what stain menthod

A

TRAP + stain

Tartate resistant acid phosphatase

160
Q

Pt is 65 yo and has Auer rods on his blood smear and a significant increase in circulating myeloblasts on peripheral smear. He is also suffering from DIC

A

Acute myelogenous leukemia (AML)

if myeloperoxidase + then it is APL ( a type of AML)

161
Q

Risk factors fo acute myelogenous leukemia

A 
alkylating chemotherapy
myeloproliferative disorders
Down syndrome
APL: t(15,17)
Responds to all-trans retinoic acid (vitamin A)
differentiation of promyelocytes
162
Q

Dysregulated production of mature and maturing granulocytes and splenomegaly. It may accelerate to AML or ALL “blast crisis”. Patient has very low Leukocyte Alkaline Phosphatase (LAP)

A

Chronic myelogenous leukemia

philadelphia chromosome t(9,22) BCR-ABL

myeloid stem cell proliferation

responds to bcr-abl tyrosine kinase inhibitors - imatinib and dasatinib

163
Q

chronic myeloproliferative disorders are associated with ____ mutation

A

V617F JAK2 mutation

polycythemia vera
essential thrombocythemia
myelofibrosis

164
Q

Patient complains of feeling extremely itchy after a long hot shower. She has also noted severe, burning pain and red-blue coloration in her extremities

A

Polycythemia vera (primary)- increased RBCs

erythromelalgia (severe burning pain and red blue coloration due to episodic blood clots in vessels of the extremities) is a classic sx

drop in EPO (vs secondary polycythemia vera which has increased EPO due to endogenour or artificially)

165
Q

Patient presents with bleeding and thrombosis. Blood smear shows markedly increased number of platelets, which may be large or otherwise abnormally formed

A

Essential thrombocythemia

massive prolfieration of megakaryocytes and platelets

166
Q

Patient has massive splenomegaly and you see teardrop RBCs

A

myelofibrosis

obliteration of bone marrow with fibrosis due to increased fibroblast activity

“bone marrow is crying because its fibrosed and is a dry tap”

167
Q

Relative polycythemia

A

drop in plasma volume due to dehydration or burns

168
Q

Appropriate absolute polycythemia

A

increase in RBC mass, drop O2 sat, increased EPO levels

lung disease, congenital heart disease, high altitude

169
Q

Inappropriate absolute polycythemia

A

increased RBC mass and increased EPO levels

malignancy, hydronephrosis, due to ectopic epo secretion

170
Q

Pt is a child presenting with lytic bone lesions, skinr ash, and recurrent otitis media with a mass involving the mastoid bone. Birbeck granules are seen on EM.

A

Langerhans cell histocytosis- collective group of proliferative disorders of dendritic (langerhans) cells

cells are functionally immature and do not effectively stimulate primary T cells via antigen presentation

S-100 and CD1a

birbeck granules are the tennis rackets

171
Q

Tumor lysis syndrome

A

Release of K+ –> hyperkalemia, release of PO4 –> hyperphosphatemia, hypocalcemia due to sequesteration of PO4

this increases nucleic acid breakdown –> hyper uricemia –> acute kidney injury

tx with aggressive hydration, allopurinol, rasburicase

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