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USMLE Step 1 > Hematology-Oncology > Flashcards

Hematology-Oncology Flashcards

0
Q

Platelet

A

From megakaryocyte
Not enough or dysfunction-petechiae
1/3 stored in spleen
Contains dense granules (ADP, Ca), alpha granules (vWF, fibrinogen)

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1
Q

WBCs, high to low in terms of percentage

A 
Neutrophils
Lymphocytes
Monocytes
Eosinophils
Basophils

“Neutrophils like making everything better”

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2
Q

Leukocyte

A

Granulocyte, mononuclear cells. 4000-10000/microliter.

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3
Q

Neutrophil

A

Multilobed nucleus. Lysosomes contain hydrolytic enzymes, lysozyme, myeloperoxidase, lactoferrin.

Multisegmented=folate, B12 def

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4
Q

Monocyte

A

Kidney shaped nucleus. Become macrophages in tissues.

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5
Q

Macrophage

A

Long tissue life. APC.

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6
Q

Eosinophil

A

Bilobed nucleus. Helminthic infections. Down regulate allergic reactions through histaminase. Phagocytic.

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7
Q

Basophils

A

Bilobate nucleus. Allergic reaction. Contain heparin, histamine, leukotrienes.

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8
Q

Mast cell

A

Allergic reaction. Degranulate-histamine, heparin, eosinophil chemo tactic factors. Type 1 hypersensitivity.

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9
Q

Dendritic cell

A

Main inducers of primary Ab response

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10
Q

Lymphocytes

A

Round densely stained nucleus, small pale cytoplasm. B and T.

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11
Q

B lymphocyte

A

Humoral immunity. Produce Abs.

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12
Q

T lymphos

A

Cellular immune response. Regulate B lymphos and macrophages.

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13
Q

Plasma cells

A

Off center nucleus, clock face chromatin. B cells differentiate into these.
Multiple myeloma is a plasma cell neoplasm.

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14
Q

Vitamin K

A

Cofactor in synthesis of II, VII, IX, X, C, S

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15
Q

Epoxide reductase

A

Activates vitamin K

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16
Q

Warfarin

A

Inhibits epoxide reductase

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17
Q

tPA

A

Degrades plasminogen into plasmin

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18
Q

Antithrombin

A

Inactivates II, VII, IX, X, XI, XII

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19
Q

vWF

A

Binds to expose collagen after endothelial damage. Platelets then bind to vWF. Eventually forms platelet plug.

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20
Q

Abciximab

A

Inhibits GpIIb/IIIa

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21
Q

Clopidogrel

A

Inhibit ADP-induced expression of GpIIb/IIIa

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22
Q

Increased ESR

A

Infections, inflamm disease, malignancy, UC, pregnancy

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23
Q

Decreased ESR

A

Polycythemia, sickle cell, CHF, microcytosis, hypofibrinogenemia

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24
Q

Bite cell

A

G6PD deficiency

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25
Q

Spur cell

A

Liver disease, abetalipoproteinemia

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26
Q

Elliptocyte

A

Hereditary elliptocytosis

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27
Q

Ringed sideroblasts

A

Sideroblastic anemia

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28
Q

Schistocyte, helmet cell

A

DIC, TTP/HUS, traumatic hemolysis

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29
Q

Spherocyte

A

Hereditary spherocytosis, autoimmune hemolysis

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30
Q

Teardrop cell

A

Bone marrow infiltration

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31
Q

Heinz bodies

A

Alpha thalassemia, G6PD def

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32
Q

Howell jolly bodies

A

Functional hyposplenia, asplenia

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33
Q

Microcytic anemias

A 
Iron deficiency
ACD
Thalassemias
Pb poisoning
Sideroblastic anemia
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34
Q

Non-hemolytic normocytic anemias

A

ACD, aplastic anemia, kidney disease

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35
Q

Megaloblastic macrocytic anemias

A

Folate def

B12 def

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36
Q

Non-megaloblastic, macrocytic anemias

A 
Liver disease
alcoholism
reticulocytosis
metabolic disorders
drugs
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37
Q

Plummer-Vinson syndrome

A

Iron deficient anemia
Esophageal web
Atrophic glossitis

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38
Q

Hb Barts

A

4 gene deletion alpha globin. Incompatible with life.

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39
Q

HbH

A

3 gene deletion alpha globin.

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40
Q

Beta thalassemia minor

A

B chain underproduced. Usu asymptomatic. Increased HbA2.

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41
Q

B thal major

A

B chain absent. Severe anemia requiring transfusions. Marrow expansion (“crew cut”) on skull XR. Increased HbF.

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42
Q

Pb poisoning

A

Lead lines on gingivae
Abdo colic
Wrist and food drop
Encephalopathy

Tx with EDTA, or succimer in kids

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43
Q

Sideroblastic anemia

A

X linked or alcohol or lead.
If creased iron, ferritin
Tx with pyridoxine

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44
Q

Folate def

A

Hypersegmented neutrophils, glossitis. Decreased homocysteine but normal methylmalonic acid.

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45
Q

B12 def

A

Hypersegmented neutrophils, glossitis, increased homocysteine AND methylmalonic acid

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46
Q

Intravascular hemolysis

A

Decreased haptiglobin
Increased LDH
Hb in urine

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47
Q

Extravascular hemolysis

A

Increased LDH, UBC (jaundice)

48
Q

Causes aplastic anemia

A

Radiation or drugs (benzene, chloramphenicol, alkylation agents, antimetabolites)
Viral agents
Fanconi anemia
Idiopathic

49
Q

G6PD def

A

X linked
Increased RBC susceptibility to oxidant stress…hemolysis
Back pain, hemoglobinuria few days later

Heinz bodies and bite cells

50
Q

Paroxysmal nocturnal hemoglobinuria

A

Complement mediated RBC lysis. Increased urine hemosiderin.

51
Q

Pyruvate kinase deficiency

A

Hemolytic anemia in newborn

52
Q

Sickle cell

A 
Crew cut on skull XR
Complications-
   1. Aplastic crisis-parvovirus B19
   2. Autosplenectomy-encapsulated bacteria
   3. Splenic sequestration crisis
   4. Salmonella osteomyelitis
   5. Painful vaso-occlusive crisis...acute chest syndrome
   6. Renal papillary necrosis
53
Q

Porphyrias

A

Defective heme synth…leads to accum of heme precursors.

54
Q

Acute intermittent porphyria

A

No porphobilinogen deaminase.
Buildup of porphobilinogen, delta-ALA, uroporphyrin

Red wine colored urine
Painful abdo
Neuro-psych sxs

55
Q

Porphyria cutanea tarda

A

No uroporphyrinogen decarboxylase
Buildup of uroporphyrin

Tea colored urine
Photosensitivity

56
Q

Pb poisoning-enzymes

A

No ferrocene lactase and ALA dehydratase

Buildup of protoporphyrin

57
Q

Major site hematopoiesis in early embryo life

58
Q

Fetal Hb

A

Does not bind 2,3-BPG, so has higher oxygen affinity

59
Q

Plasma

A

55% total blood volume

60
Q

Serum

A

Plasma without clotting factors

61
Q

Corticosteroids affect on blood

A

Inhibit PMN migration from circulation to periphery, thus benign leukocytosis

62
Q

RBC energy source

A

90% glucose anaerobically degraded to lactate. Ernest through HMP shunt

63
Q

Reticulocytes

A

Immature RBCs. Polyribosomes. Increased in blood loss, hemolytic anemia.

64
Q

NK cell

A

Cytotoxic lymphocyte. Contains perforin, proteases. Target and kill cells lacking MHC1 (incl tumor derived cells, virus infected cells)

65
Q

Causes eosinophilia

A 
"NAACP"
Neoplasia
Asthma 
Allergic processes
Collagen vascular disorders
Parasites
66
Q

Dendritic cells

A

Main inducers of primary Ab response

67
Q

B thalassemia minor

A

Reduced B chains

68
Q

B thal major

A

Absent B chain.

Reduced RBC lifespan and apoptosis of precursors.
Anemia, splenomegaly, hemosiderosis from repeat transfusions, skeletal deformities (“crew cut” on skull XR)
Mediterranean

69
Q

A thalassemia

A

Africa, Asian populations. Lack of alpha globin.

70
Q

HbBarts

A

Gamma tetramers, four gene deletion alpha globin. Fatal.

71
Q

Alpha thalassemia trait

A

2 gene deletion alpha globin. Minimal anemia

72
Q

HbH

A

3 gene deletion. High oxygen affinity. Anemia disproportionate to amt Hb.

73
Q

Schilling test

A

Tests if B12 def due to lack intrinsic factor

74
Q

Neuro problems in B12 def

A

Demyelination of dorsal and lateral columns

Ataxia, hyper reflexes, decreased positional and vibration sensation

75
Q

G6PD

A

Protects RBCs from oxidant stress,

76
Q

G6PD def

A

X linked. Black, middle eastern populations. Associated with resistance to malaria. Hemolysis from oxidant stress eg drugs, infections, favs beans.
History of neonatal jaundice and cholelithiasis
Episodic fatigue, pallor, symptomatic anemia
Heinz bodies, bite cells

77
Q

Hereditary spherocytosis

A

AD def of spectrin, ankyrin. Increased RBC fragility. Spherocytes.
Anemia, splenomegaly, jaundice, maybe cholelithiasis.

78
Q

Immunohemolytic anemias

A

Coombs positive

79
Q

Erythroblastosis fetalis

A

Destruction of fetal Hb. Fetal hemolytic anemia.

80
Q

Sickle cell

A

Sickling when deoxygenated..splenic destruction.
Blood hyperviscous.
Spleen particularly vulnerable since no collateral supply…auto splenectomy by 6 years.

HOWELL JOLLY BODIES.

81
Q

“Crew cut” on skull XR

A

Extra medullary hematopoiesis.

Sickle cell, B thal.

82
Q

Paroxysmal nocturnal hemoglobinuria

A

Acquired. Lysis by endogenous complement.

83
Q

HUS (Hemolytic Uremic Syndrome)

A

Hemolytic anemia, AKF, thrombocytopenia. Mostly kids. Most cases preceded by infectious diarrhea. Commonest cause AKF in kids.

84
Q

LDH

A

Extensive in body tissues, e.g. RBCs, myocardium. Released during tissue damage, thus marker of common injuries and disease

85
Q

Micropathic hemolytic anemia

A

Mechanical trauma by narrowed vessels. DIC, TTP, HUS, SLE, malignant HTN

86
Q

Aplastic anemia

A

Severe anemia, neutropenia, thrombocytopenia. Failure/destruction of multipotent myeloid stem cells.

Idiopathic, radiation, benzene, drugs (chloramphenicol, sulfonamides, Alkylating agents, antimalarials, antimetabolites), viruses (parvovirus B19, EBV, HIV, HCV), Fanconi anemia

87
Q

Thrombocytopenia

A

Purpura,mucosal bleeding, petechiae

88
Q

Porphyrias

A

Defects in heme synthesis,resulting in accumulation of heme intermediates

89
Q

Acute intermittent porphyria

A

Increased porphobilinogen and ALA
Causes myelin degeneration
Dark foul smelling urine, hallucinations, blurred vision, gross neuro manifestations eg foot drop

90
Q

Porphyria cutanea tarda

A

Increased uroporphyrin, Fe, transferrin
Accumulation of Fe leads to siderosis which affects liver
Photosensitivity and cutaneous bullae.
Tx with low dose antimalarials

91
Q

Hemophilia

A

Intrinsic pathway defect. Increased PTT.
A-VIII
B-IX.

92
Q

Ehlers danlos

A

Inherited collagen and/or elastin abnormality. Decreased vessel wall strength, hyper elasticity of skin, joint abnormality, tissue fragility

93
Q

PT

A

Extrinsic and common pathway. Factors V, VII, X, prothrombin, fibrinogen

94
Q

PTT

A

Intrinsic. Increased with heparin

95
Q

Waldenstrom macroglobulinemia

A

Hyperviscous blood damages vessels

96
Q

Idiopathic thrombocytopenic purpura

A

Thrombocytopenia with normal or increased megakaryocytes. Antiplatelet Abs attach to platelets…removed by spleen.

97
Q

TTP

A

ADAMTS13 def….which normally degrades vWF. Thus, platelet aggregation. Adult females.

98
Q

Von Willebrand disease

A

Most common inherited bleeding disorder. AD. vWF normally carries factor VIII and mediates platelet adhesion.

99
Q

Bernard Soulier

A

AR. Lack platelet surface GpIb (needed for platelet adhesion).

100
Q

ALL

A

<15 years. Most responsive to tx. Bone marrow replaced by loads of lymphoblasts

Association with Downs.

101
Q

CLL

A

> 60 years. Oft a symptomatic. Smudge cells.

102
Q

AML

A

Auer rods. Loads of myeloblasts. T(15;17) subtype= M3 or APL subtype

103
Q

CML

A

Philedelphia chromosome t(9;22).

Bcr-abl

104
Q

Langerhans cell histiocytosis

A

Proliferation of langerhans cells. Birbeck granules “tennis rackets” on electron microscopy.
Immature cells that do not efficiently stimulate T lymphos.

105
Q

JAK2 mutations

A

Polycythemia Vera, essential thrombocytosis, myelofibrosis

106
Q

JAK2

A

Involved in Hematopoietic growth factor signaling

107
Q

Polycythemia vera

A

Increased RBCs, WBCs, platelets

108
Q

Myelofibrosis

A

Decreased RBCs

109
Q

Essential thrombocytosis

A

Increased platelets

110
Q

Bcr-abl

A

Increases cell division and decreases apoptosis

111
Q

LMWH, eg enoxaparin

A

Act more on Xa. Better bioavailability, 2-4x longer half life than regular heparin.

112
Q

Intravascular hemolysis

A

Hemoglobinemia, hemoglobinuria, decreased serum haptoglobin

Result of complement fixation, mechanical injury, or toxins

113
Q

Extravascular hemolysis

A

Splenomegaly

Result of RBC injury, Ab attachment, or decrease in deformability.

114
Q

Mixing study

A

Pt’s blood mixed with all factors nec for clotting. If bleeding time corrects, then it is a clotting factor deficiency. If it does not correct, it is an Ab to one of the clotting factors.

115
Q

Hereditary hemorrhagic telangiectasia

A

AD mutation leading to dilated tortuous vessels with thin walls in skin and mucous membranes

116
Q

Bleeding time

A

Prolonged if platelet abnormality

117
Q

Liver disease

A

Prevents production all coag factors (except vWF). Thus, hypersplenism and thrombocytopenia.
Can actuaries bleeding into joints and easy bruising.

USMLE Step 1 (15 decks)

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