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Step 1 > HemeOnc > Flashcards

HemeOnc Flashcards

1
Q

Stain for erythrocytes

A

Blue on Wright-Giemsa stain - Stains residual reticular rRNA

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2
Q

GpIb and GpIIb/IIIa

A 
GpIb = vWF receptor
GpIIb/IIIa = Fibrinogen receptor
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3
Q

Platelet dense granules and a-granules

A 
Dense = ADP, Ca
a-granules = vWF, Fibrinogen, Fibronectin
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4
Q

Neutrophil Specific and Azurophilic granules

A

Specific = LAP, Collagenase, Lysozyme, Lactoferrin

Azurophilic = Proteinases, Acid phosphatase, Myeloperoxidase, b-Glucuronidase

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5
Q

Neutrophilic chemotactic agents

A 
C5a
IL-8
LTB4
Platelet activating factor
Kallikrein
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6
Q

Hypersegmented neutrophils (6+ lobes)

A

Vit B12/Folate deficiency

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7
Q

Kidney shaped nucleus

“Frosted glass” cytoplasm

A

MONOCYTE

Becomes macrophage in tissues

Note - Lipid A from LPS binds CD14 on macrophages and leads to septic shock

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8
Q

Produced by eosinophils

A

Major basic protein (helminthotoxin)

Histaminase

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9
Q

Causes of eosinophilia (5)

A 
Neoplasia
Asthma
Allergy
Chronic adrenal insufficiency
Parasites
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10
Q

Produced by basophils

A

Heparin
Histamine
Leukotrienes

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11
Q

Drug preventing mast cell degranulation

A

Cromolyn sodium

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12
Q

Lymphocytes with eccentric nuclei and large eccentric nucleus

A

PLASMA CELL

Normally do not circulate

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13
Q

Sites and timeline of fetal erythropoiesis

A

Yolk sac (3 - 8 weeks)
Liver (6 weeks - Birth)
Spleen (10 - 28 weeks)
Bone marrow (18 weeks - Adult)

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14
Q

Fetal to adult hemoglobin development

A

HbF (a2y2) has y replaced by b to become HbA1 (a2b2)

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15
Q

Universal donor and acceptor of plasma

A

Universal donor - AB (no IgMs)
Universal acceptor - O (both IgGs)

Note - Opposite of universal donor and acceptor of RBCs

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16
Q

Mechanism of Rh hemolytic disease of the newborn

A

Rh- mother has Rh+ pregnancy
Exposure to Rh+ blood results in anti-D IgG
Subsequent Rh+ pregnancies anti-D IgG crosses placenta

Treat with RhoGAM (anti-D IgG) during first pregnancy to opsonize Rh+ fetal erythrocytes

Note - Resulting Coombs+ anemia and hydrops fetalis is due to maternal antibodies as the infant is not yet making its own antibodies

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17
Q

Mechanism of ABO hemolytic disease of newborn - mild jaundice resolved with phototherapy or exchange transfusion and also see hyperbilirubinemia

immune hemolytic anemia due to ABO incompatibility. Infant A or B and mother is O.

A

Blood type O mother with A, B, or AB fetus makes anti-A or anti-B IgG which crosses placenta - Does not worsen with future pregnancies

Note - Type O mothers make IgG instead of IgM

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18
Q

Order of hemoglobin migration (greatest to least) on gel electrophoresis - Negatively charged so moves from Cathode (-) to Anode (+)

(“A Fat Santa Claus”)

A

HbA
HbF
HbS
HbC

Note - Replacement of Glutamic acid (-) with Valine (neutral) in HbS and Lysine (+) in HbC responsible for relative migration on gel

Note - Paradoxically, HbF has a less positive binding pocket for 2,3-BPG compared to HbA

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19
Q

Effects of Bradykinin - Converted from HMWK by Kallikrein

A

Vasodilation
Permeability
Pain

Note - HMWK also helps activate XII

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20
Q

Contact activation (intrinsic) pathway

A

Collagen, basement membrane, activated platelets exposed

XII = XIIa
XIIa + XI = XIa
XIa + IX = IXa
IXa + VIIIa + X + Ca + Phospholipid = Xa

Note - vWF carries and protects VIII

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21
Q

Tissue factor (extrinsic) pathway

A

VII + TF + Ca + Phospholipid = VIIa

VIIa + X + Ca + Phospholipid = Xa

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22
Q

Combined pathway

A

Xa + Va + II (Prothrombin) + Ca + Phospholipid = IIa
IIa (Thrombin) + I (Fibrinogen) = Ia
Ia (Fibrin) + XIIIa + Ca = Fibrin mesh

Note - IIa (Thrombin) activates V, VIII, and XIII

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23
Q

Fibrinolytic system

A

Plasminogen + tPA = Plasmin
Plasmin + Fibrin mesh = Fibrin degradation products

Note - Also destroys coagulation factors

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24
Q

Mechanism of Vit K procoagulation

Defect results in…
Normal BT
Increased PT
Increased PTT

A

Oxidized Vit K reduced by Epoxide Reductase
Reduced Vit K cofactor for y-Glutamyltransferase
y-Glutamyl Transferase activates X, IX, VII, II, C, S

Note - Neonates lack enteric bacteria which produce Vit K and can develop ICH (downset eyes, bulging fontanelle)

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25
Q

Anticoagulation cascade

A

Protein C + Thrombin-Thrombomodulin complex
Activated Protein C
Activated Protein C + Protein S cleaves Va, VIIIa

Note - Factor V Leiden is protein C resistant Va

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26
Q

Principal target of AT

A

II (Thrombin)
Xa

Note - Also X, IX, VII, II, XII, XI

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27
Q

Mechanism of platelet plug formation - primary hemostasis

Note = Unstable/easily dislodged plug stops bleeding until secondary hemostasis (coagulation cascade)

A

Endothelial damage releases Endothelin (transient vasoconstriction)

vWF binds to exposed collagen

Platelet Gp1b bind vWF at site of injury (specific), then undergo conformational change

Platelets release ADP and Ca

ADP helps adherence platelets to endothelium and induces GpIIb/IIIa expression on platelets

Fibrinogen binds GpIIb/IIIa and links platelets

Note - Decreased blood flow and TXA2 (platelets) cause further aggregation, while increased blood flow and PGI2/NO (endothelial cells) inhibit aggregation

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28
Q

Source of vWF

A

Weibel-Palade bodies of endothelial cells

a-granules of platelets

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29
Q

Coagulation factors produced by endothelial cells

A 
vWF
VIII
Thromboplastin (II to IIa)
tPA
PGI2

Note - Also express thrombomodulin membrane protein for activation of Protein C

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30
Q

Acanthocyte (spur cell) - spiny surface

A

Liver disease

Abetalipoproteinemia (B-48, B-100)

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31
Q

Basophilic stippling

A

Lead poisoning
Sideroblastic anemia
Myelodysplastic syndromes

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32
Q

Dacrocyte (tear drop cells)

A

Bone marrow infiltration (e.g. myelofibrosis)

Note - From being mechanically squeezed out of the bone marrow

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33
Q

Degmacyte (bite cells)

A

G6PD deficiency

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34
Q

Echinocyte (burr cell)

Note - More uniform, smaller projections than Acanthocyte

A 
ESRD
Liver disease
Pyruvate kinase deficiency
Microangiopathic hemolytic anemia
Macroangiopathic hemolytic anemia
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35
Q

Elliptocyte

A

Hereditary elliptocytosis

Note - Mutation in Spectrin

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36
Q

Macro-ovalocyte

A 
Megaloblastic anemia (+ hypersegmented PMNs)
Marrow failure
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37
Q

Ringed sideroblasts - Stain with Prussian blue due to iron-laden mitochondria surrounding nucleus

Note - Nucleated RBC

A

Sideroblastic anemia

Note - Only present on marrow staining

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38
Q

Schistocyte - Fragments of RBCs

Note - Includes helmet cell

A 
Microangiopathic hemolytic anemias...
DIC
TTP/HUS
SLE
Malignant HTN

Macroangiopathic hemolytic anemias…
Aortic stenosis
Prosthetic valves

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39
Q

Causes of sickling

A

Dehydration
Deoxygenation
High altitude

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40
Q

Spherocytes

A

Hereditary spherocytosis

Drug/Infection induced hemolytic anemia

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41
Q

Target cell

A

HbC disease
Asplenia
Liver disease
Thalassemia

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42
Q

Heinz bodies - Peripheral inclusions of oxidized and denatured hemoglobin

A

G6PD deficiency

hemolysis occurs following exposure to oxidants or infections

Oxidation of Hb SH to SS bonds allows precipitation and subsequent phagocytic damage (e.g. bite cells)

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43
Q

Howell-Jolly bodies - basophilic nuclear remnants normally removed by splenic macrophages

A

Asplenia

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44
Q

Microcytic anemias

A 
Iron deficiency
a-thalassemia
b-thalassemia
Lead poisoning
Sideroblastic anemia
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45
Q

Fatigue
Conjunctival pallor
Pica
Spoon nails

Decreased Iron
Decreased Ferritin
Increased TIBC/Transferrin
Decreased Transferrin Saturation (Fe/TIBC)

A

IRON DEFICIENCY ANEMIA

Microcytosis with hypochromia

Note - Increased TIBC and Decreased Transferrin Saturation seen in OCP use but Fe levels are normal

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46
Q

Various presentations of a-thalassemia

A

4 allele deletion - Hb Barts (y4) with hydrops fetalis
3 allele deletion - HbH disease (b4)
2 allele deletion - Mild clinical anemia
1 allele deletion - Clinically silent

Note - Trans deletions (opposite chromosomes) more common in African Americans

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47
Q 
Target cells
Anisopoikilocytosis
Increased HbF
Increased HbA2 (a2d2)
Hepatosplenomegaly (extramedullary hematopoiesis)
Crew cut on skull XR/Chipmunk facies
Secondary hemochromatosis (transfusions)
B19 aplastic crisis

Note - Becomes symptomatic as HbF declines (6 months)

A

B-THALASSEMIA MAJOR

Point mutation in splice sites and promoter sequences leads to absent b chain

Microcytic, hypochromic anemia

Note - HbA1c readings artificially high due to HbA1 underproduction

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48
Q

Diagnosis of b-thalassemia minor - underproduction rather than absence of b globulin

A

HbA2 (a2d2) > 3.5% - usually clinically silent

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49
Q 
Mechanism of...
Burton lines on long bone metaphyses and gingiva
Encephalopathy
Abdominal colic
Wrist and foot drop
Microcytic anemia with normal Fe studies
A

LEAD POISONING

Inhibited heme synthesis due to deficient…
d-ALA dehydratase (cytoplasmic)
Ferrochelatase (mitochondrial)

Increased RBC d-ALA or Protoporphyrin

Inhibition of rRNA degradation leading to aggregate formation - Basophilic stippling (peripheral) and ringed sideroblasts (marrow)

Treat with…
Dimercaprol/EDTA
Succimer chelation in children

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50
Q 
Increased Iron
Increased Ferritin
Normal/Decreased TIBC
Ringed sideroblasts in marrow
Basophilic stippling in peripheral smear
A

SIDEROBLASTIC ANEMIA

X-linked d-ALA Synthase (mitochondrial) gene mutation results in defective heme synthesis

Non-genetic causes include...
Vit B6 deficiency (Isoniazid)
Copper deficiency
Lead
Alcohol

Treat with…
Pyridoxine - Cofactor for d-ALA synthase

Note - Converts Glycine, Succinyl-CoA, and B6 to d-ALA

51
Q

Macrocytic anemias

Note - Megaloblastic (delayed DNA synthesis) also presents with Hypersegmented neutrophils and Glossitis

A 
Megaloblastic (> 115):
Folate deficiency
B12 deficiency
Orotic aciduria
NO (inhibits Methionine Synthase)
Non-megaloblastic:
Liver disease
Alcoholism
Hypothyroidism
Diamond-Blackfan anemia
Myelodysplastic syndromes

Note - Increased cell turnover (e.g. SCD) causes folate deficiency

52
Q 
Rapid-onset anemia within first year
Short stature
Craniofacial abnormalities
Triphalangeal thumbs
Increased HbF
Normal total Hb
A

DIAMOND-BLACKFAN ANEMIA

Intrinsic defect in erythroid progenitor cells

53
Q 
Indirect hyperbilirubinemia
Decreased haptoglobin
Increased LDH
Hemoglobinuria
Hemosiderinuria
Urobilinogen
Schistocytes, Reticulocytes on peripheral smear
A

INTRAVASCULAR HEMOLYSIS

Mechanical hemolysis
Paroxysmal nocturnal hemoglobinuria
Microangiopathic hemolytic anemia

54
Q 
Indirect hyperbilirubinemia
Increased LDH
Urobilinogen
No hemoglobinuria
No hemosiderinuria
Spherocytes on peripheral smear
A

EXTRAVASCULAR HEMOLYSIS

55
Q

Normocytic, nonhemolytic anemia
Decreased Iron
Increased Ferritin
Decreased TIBC/Transferrin

Note - Normocytic, nonhemolytic anemias have normal or decreased Retic counts

A

ANEMIA OF CHRONIC DISEASE

Increased Hepcidin from liver binds Ferroportin on intestinal mucosa and macrophages decreasing Iron release from macrophages and absorption from the gut

Treat with EPO

56
Q 
Fatigue
pallor
Purpura
Mucosal bleeding
Petechiae
Infection
Severe normocytic, nonhemolytic anemia
Leukopenia
Thrombocytopenia
Decreased reticulocyte count
Increased EPO
Normal cell morphology
Hypocellular bone marrow with fatty infiltration
A

APLASTIC ANEMIA

Caused by...
Radiation
Drugs (Chloramphenicol, Sulfonamide, Carbamazepine)
Toxins (Benzene, Solvents)
Viral (B19, EBV, HIV)
Fanconi anemia
Autoimmune
Treat with...
Immunosuppression
Transfusion
GM-CSF
Bone marrow allograft
57
Q 
Aplastic anemia
Short stature
Cafe-au-lait spots
Thumb and Radius defects
Increased incidence of cancer
A

FANCONI ANEMIA

DNA cross-link repair defect

58
Q 
Extravascular hemolysis (e.g. viral illness)
Aplastic crisis (B19)
Splenomegaly
Osmotic fragility+
Elevated MCHC
A

HEREDITARY SPHEROCYTOSIS

Autosomal dominant defect in proteins interacting with RBC membrane (e.g. Spectrin, Ankyrin) - premature removal by spleen

Treat with splenectomy

59
Q 
Episodic...
Extravascular and intravascular hemolysis
Back pain
Hemoglobinuria
Heinz bodies
Bite cells
A

G6PD DEFICIENCY

X-linked recessive defect in G6PD - Prevents reduction of NADP to NADPH which is used to reduce Glutathione

60
Q

Extravascular hemolysis in a newborn
Splenomegaly (red pulp hyperplasia)
Echinocytes

A

PYRUVATE KINASE DEFICIENCY

Autosomal recessive mutation resulting in decreased ATP leading to rigid RBCs

Note - No mitochondria in RBC so all energy must come from lactate

61
Q

Splenomegaly
Extravascular hemolysis
Hemoglobin crystals in RBCs
Target cells

A

HBC DISEASE

Glutamic acid to Lysine mutation in b-globulin

Note - HbSC milder disease than HbSS

62
Q 
Coombs- intravascular hemolysis
Pancytopenia
Venous thrombosis
CD55/59- RBCs
Increased incidence of leukemia
A

PAROXYSMAL NOCTURNAL HEMOGLOBINURIA

Impaired synthesis of GPI anchor for decay-accelerating factor - Deficient protection of RBC membrane from complement lysis

Treat with Eculizumab (terminal complement inhibitor)

63
Q 
Intravascular and extravascular hemolysis
Crew-cut on skull XR (marrow expansion)
Possibly Howell-Jolly bodies
Absent HbA
Increased HbF
A

SICKLE CELL ANEMIA

Glutamic acid to Valine mutation results in polymerization of deoxygenated HbS

Treat with…
Hydroxyurea (increases HbF)
Hydration

Note - Newborns initially asymptomatic due to high HbF

64
Q

Complications of SCD

A 
Aplastic crisis (B19)
Splenic sequestration/congestion (infancy)
Splenic atrophy/fibrosis (childhood)
Encapsulated infections
Renal papillary necrosis
Medullary infarcts (microhematuria)
Pigmented gallstones
Vaso-occlusive crises (Dactylitis, Priapism, ACS, Avascular necrosis, Stroke)
65
Q

Warm autoimmune hemolytic anemia

A

Coombs+ IgG mediated…

SLE
CLL
a-Methyldopa

66
Q

Cold autoimmune hemolytic anemia

A

Coombs+ IgM and Complement mediated…

CLL
Mycoplasma
EBV (Mononucleosis)

Note - May cause painful blue fingers and toes with cold exposure

67
Q

Normocytic anemias

A

Non-hemolytic:

ACD
CKD
Aplastic anemia

Hemolytic:

Intrinsic - Hereditary spherocytosis, G6PD, Pyruvate Kinase, HbC, Paroxysmal Nocturnal Hemoglobinuria, SCD

Extrinsic - AIHD, Micro/Macroangiopathic, Infections

68
Q

Increased Iron
Increased Ferritin
Decreased TIBC/Transferrin
Increased Transferrin Saturation

A

Hemochromatosis

69
Q

Hematologic effects of Corticosteroids

A

Neutrophilic - Decreased adhesion and extravasation
Leukopenia - Increased apoptosis
Eosinopenia - Sequestration in lymph nodes

70
Q

Left shift

Immature RBCs

A

LEUKOERYTHROBLASTIC REACTION

Severe anemia
Marrow fibrosis
Tumor taking up space in marrow

71
Q

Painful abdomen
Port-wine colored urine
Polyneuropathy
Psychological disturbances

Precipitated by…
Drugs (Cytochrome P-450 inducers)
Alcohol
Starvation

A

ACUTE INTERMITTENT PORPHYRIA

Porphobilinogen Deaminase (cytoplasmic) deficiency results in accumulation of…

d-ALA
Porphobilinogen

Treat by inhibiting ALA synthase with Glucose/Heme

Note - Also paradoxically Coproporphyrinogen (urine)

72
Q

Blistering cutaneous photosensitivity

Tea-colored urine

A

PORPHYRIA CUTANEA TARDA

Uroporphyrinogen Decarboxylase (cytoplasmic) deficiency results in accumulation of Uroporphyrinogen (tea-colored urine)

73
Q 
Child with...
Nausea
Vomiting
Gastric bleeding
Lethargy
Opacity in stomach on CXR

Note - Long term may result in scarring and GI obstruction

A

IRON POISONING

Cell death due to peroxidation of membrane lipids

Treat with…
IV Deferoxamine
Oral Deferasirox
Dialysis

74
Q

Factors tested by PT

Note - INR is normalized PT

A

Extrinsic and Common pathways…
VII, X, V, II, I

Note - Follow PT/INR with Warfarin use

75
Q

Factors tested by PTT

A

Intrinsic and Common pathways…

All except VII and XIII

76
Q

Deficiencies and treatment of…
Hemophilia A
Hemophilia B
Hemophilia C

Note - All present with isolated elevated aPTT

A

VIII (X-linked recessive)
IX (X-linked recessive)
XI (Autosomal recessive)

All receive Desmopressin and appropriate factor

77
Q 
Microhemorrhage...
Bleeding mucous membranes
Epistaxis
Petechiae
Purpura
Increased BT
Decreased PC
A

Platelet disorders

Includes...
Bernard-Soulier
Glanzmann thrombasthenia
HUS
ITP
TTP
78
Q

Defect in platelet plug formation - large platelets

A

BERNARD-SOULIER SYNDROME

autosomal recessive - deficient GpIb leads to impaired platelet-vWF adhesion (GpIb normally bind vWF) and also diminished production of platelets by megakaryocytes in bone marrow

presents with petechia, purpura, menorrhagia, and prolonged bleeding with trauma and surgery

decreased platelet count, presence of giant platelets, prolonged bleeding time, and normal PT and PTT

abnormal platelet aggregation with ristocetin

79
Q

Defect in platelet plug formation - no platelet clumping on smear

Note - Platelet count usually normal

A

GLANZMANN THROMBASTHENIA

Deficient GpIIb/IIIa leads to impaired platelet-platelet aggregation

80
Q 
Child with...
Diarrhea (bloody)
Thrombocytopenia
Microangiopathic hemolytic anemia with schistocytes
Acute renal failure
decreased hemoglobin
decreased haptoglobin
increased lactate dehydrogenase
increased unconjugated bilirubin
increased bleeding time
A

HEMOLYTIC UREMIC SYNDROME (TTP-HUS)

Shiga toxin-producing EHEC (O157:H7) leads to microthrombi formation or shigella dysenteriae

Treat with Plasmapheresis

Note - No EHEC or diarrhea in adults

81
Q

Viral illness followed by…
Petechiae
Mucosal bleeding
Increased megakaryocytes/hyperplasia on bone marrow smear

Increased BT
Normal PT
Normal aPTT
Decreased PC

A

IMMUNE THROMBOCYTOPENIA (ITP)

antibodies against platelet glycoproteins which results in platelet removal by splenic reticuloendothelial system

Anti-GpIIb/IIIa antibodies leads to splenic macrophage consumption of platelet-antibody complex

MUST rule out secondary thrombocytopenia due to lupus, viral infection, drugs etc.

acute form in children
chronic form in adults

Treat with…
Steroids
IVIG
Splenectomy if refractory

82
Q 
Fever
Thrombocytopenia
Microangiopathic hemolytic anemia
Neurologic symptoms
Renal symptoms

Schistocytes
Increased LDH
Normal PT
Normal aPTT

A

THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP)

Deficiency of ADAMTS13 (vWF metalloprotease) leads to decreased degradation of vWF multimers - Increased platelet adhesion, aggregation, and thrombosis

Treat with…
Steroids
Plasmapheresis

83
Q 
Increased BT
Normal PT
Normal/Increased aPTT
Normal platelet count
No aggregation with ristocetin cofactor assay

Note - PTT may be normal

A

VON WILLEBRAND DISEASE

Autosomal dominant deficiency in vWF leads to elevated PTT (carries/protects VIII), and defect in platelet plug formation (GpIb-vWF adhesion)

Treat with Desmopressin - Releases vWF from endothelial cells

note:vWf is produced by endothelial cells and megakaryocytes

84
Q 
Increased BT
Increased PT
Increased PTT
Decreased platelets/Thrombocytopenia
Decreased Fibrinogen
Increased D-dimer (fibrin degradation product)
Decreased V, VIII
Schistocytes
A

DISSEMINATED INTRAVASCULAR COAGULATION

Is due to massive tissue factor(thromboplastin) release which generates thrombin and initiates the clotting cascade

Caused by...
Gram- sepsis
Trauma
Obstetric complications
Acute pancreatitis
Malignancy
Nephrotic syndrome
Transfusion
85
Q

Diminished increase in PTT with Heparin administration

A

ANTITHROMBIN DEFICIENCY

May be inherited or acquired (renal failure) - leads to decreased inhibition of IIa and Xa

86
Q

Skin necrosis with hemorrhage after administration of Warfarin

A

PROTEIN C/S DEFICIENCY

87
Q

Prothrombin gene mutation

A

Mutation in 3’ UTR - Increased production and venous clots

88
Q

Mechanism of hypocalcemia following transfusion

A

Citrate in packed RBCs is a Ca chelator

89
Q

Ingredients of Cryoprecipitate

A 
vWF
VIII
XIII
Fibrinogen
Fibronectin
90
Q

Used to treat…
DIC
Cirrhosis
Immediate Warfarin reversal

A

FRESH FROZEN PLASMA

91
Q

B cell neoplasm with…
Multiple lymph node involvement
Noncontiguous spread
Extranodal involvement

Note - May be associated with HIV or autoimmune disease

A

NON-HODGKINS LYMPHOMA

92
Q 
Young adult or >55 y/o (bimodal) with...
Low-grade fever
Night sweats
Weight loss
Localized, single group of nodes
"Owl Eyes" cells

Note - Associated with EBV

A

HODGKIN’S LYMPHOMA

Contiguous spread

Relatively good prognosis

93
Q

Surface markers for “Owl Eyes”

Note - Necessary but not sufficient for diagnosis of Hodgkin’s lymphoma

A

REED-STERNBERG CELLS

B-cell origin
CD15
CD30

94
Q

Non-Hodgkin’s lymphoma…
Associated with EBV
Jaw lesions (Africa) or pelvic/abdominal lesions

“Starry sky” - Sheet of lymphocytes with interspersed “tingible body” macrophages

A

BURKITT LYMPHOMA

t(8;14) - Translocation of c-myc and heavy-chain Ig leads to activation of c-myc

Note - Heavy-chain Ig constitutively expressed so translocation near this gene causes overexpression

95
Q

Most common type of non-Hodgkin’s lymphoma in adults

A

DIFFUSE LARGE B-CELL LYMPHOMA

96
Q

Non-Hodgkin’s lymphoma with…
Painless waxing and waning lymphadenopathy
Small cleaved cells, large cells, or mixture

A

FOLLICULAR LYMPHOMA

t(14;18) - Translocation of heavy-chain IgG and Bcl-2

Note - Bcl-2 inhibits apoptosis and so this translocation promotes cell proliferation

97
Q

Aggressive non-Hodgkin’s’ lymphoma presenting in adult males

A

MANTLE CELL LYMPHOMA

t(11;14) - Translocation of cyclin D1/bcl-1 (G1 to S) and heavy-chain IgG

also seen in multiple myeloma

98
Q 
Non-Hodgkin's lymphoma with...
Confusion
Memory loss
Seizures
Mass lesions on MRI
A

PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA

Considered an AIDS defining illness

99
Q 
Non-Hodgkin's lymphoma with...
T-cell origin
Cutaneous lesions
Lytic bone lesions
Hypercalcemia

Note - Associated with IVDU

A

ADULT T-CELL LYMPHOMA

Caused by HTLV

100
Q

Non-Hodgkin’s lymphoma with…
T-cell origin
Skin patches and plaques
CD4+ cells with “cerebriform” nuclei

A

MYCOSIS FUNGOIDES (CUTANEOUS T CELL LYMPHOMA)

May progress to Sezary syndrome (T cell leukemia)

101
Q
  • Hypercalcemia
  • Bone pain
  • Back pain
  • Lytic bone lesions leading to pathologic fractures
  • Elevated ESR
  • Serum monoclonal spike
  • Renal involvement (Bence Jones Ig light chains)
  • Increased infections
  • Primary amyloidosis
  • Anemia

Rouleaux RBC formation
“Fried egg” plasma cells
Intracytoplasmic inclusions (Ig)
M spike (y) on serum protein electrophoresis

A

MULTIPLE MYELOMA

Monoclonal plasma cell cancer in marrow -that produce excess amounts of immunoglobulin (usually IgG or IgA) –> M spike

Excess free kappa and lambda chains may be filtered into urine (bence jones proteins)

Note - Isolated M spike without CRAB symptoms is MGUS

102
Q

Cause of hyperviscosity syndrome…
Blurred vision
Raynaud phenomenon

A

WALDENSTROM MACROGLOBULINEMIA

M spike of IgM - no CRAB findings

103
Q 
Defects in cell maturation of all nonlymphoid lineages...
Bleeding (thrombocytopenia)
Fatigue (anemia)
Recurrent infections (myelocytes)
Also decreased mast cells

Ringed sideroblasts on marrow smear

Note - Myelocytes include basophils, eosinophils, neutrophils, macrophages

A

MYELODYSPLASTIC SYNDROMES

Caused by...
De novo mutations
Radiation
Chemotherapy
Benzene

Note - May transform to AML

104
Q

Neutrophils with bilobed nuclei - typically seen after chemotherapy

A

PSEUDO-PELGER-HUET ANOMALY

105
Q 
Child with...
Anemia
Thrombocytopenia
Neutropenia
Increased blasts on peripheral smear
TdT+ (pre-T, pre-B)
Mostly CD10+ (pre-B)

Note - Associated with Down’s syndrome

A

ACUTE LYMPHOBLASTIC LEUKEMIA/LYMPHOMA (ALL)

t(12;21) has a better prognosis
Spreads to Testes and CNS

T-Cell ALL may present as mediastinal mass - SVC syndrome, dysphagia, dyspnea/stridor

106
Q

Elderly with…
Autoimmune hemolytic anemia
Smudge cells on peripheral smear
CD19+ CD20+ (B-cell) with CD5+

A

CHRONIC LYMPHOCYTIC LEUKEMIA (SMALL LYMPHOCYTIC LEUKEMIA)

Treat with Alemtuzumab or Rituximab

107
Q

CLL/SLL transformation into aggressive lymphoma - most commonly DLBCL

A

RICHTER TRANSFORMATION

108
Q 
Adult male with...
Massive splenomegaly
Marrow fibrosis (dry tap)
Filamentous projections on LM
Stains TRAP+
CD20+ (B-cell)
A

HAIRY CELL LEUKEMIA

Treat with…
Cladribine
Pentostatin

109
Q

Anemia
Thrombocytopenia
Neutropenia
Myeloblasts (basophilic cytoplasm, almost no nuclei)
Auer rods - Myeloperoxidase+ cytoplasmic inclusions that are azurophilic and formed by the fusion of primary granules
Pancytopenia
DIC

Associated with…
Prior exposure to alkylating chemotherapy or radiation
Myeloproliferative disorders
Down syndrome

Note - May present with DIC

A

ACUTE PROMYELOCYTIC LEUKEMIA (M3-AML)

t(15;17) - Responds to all-trans retinoic acid inducing differentiation of promyelocytes

PML/RARalpha gene produced

retinoic acid receptor alpha gene on chromosome 17

110
Q 
Adult or elderly with...
Splenomegaly
Anemia
Leukocytosis with left shift (leukocyte differentiation with a left shift)
Thrombocytosis

Maturing granulocytes (vs blasts in AML)
Low leukocyte alkaline phosphatase (LAP)
May progress to AML or ALL (blast crisis)

Note - High LAP in leukemoid reaction

A

CHRONIC MYELOGENOUS LEUKEMIA (CML)

Philadelphia chromosome t(9;22) BCR-ABL1 that encodes a constitutively active tyrosine kinase

Responds to bcr-abl tyrosine kinase inhibitor (Imatinib)

Note - JAK2 mutation negative unlike other chronic myeloproliferative disorders

111
Q

Child with…
Lytic bone lesions
Skin rash
Recurrent OM with mastoid bone mass

Birbeck (“tennis racket”) granules/rods on EM
S-100+ (mesodermal origin)

A

LANGERHANS CELL HISTIOCYTOSIS

112
Q

Intense itching after hot shower
Erythromelalgia (burning pain, red-blue coloration) due to episodic blood clots in vessels of extremities

Increased RBCs/Hct
Decreased EPO
Leukocytosis
Thrombocytosis

A

POLYCYTHEMIA VERA

Always JAK2+

Treat with ASA

113
Q

Bleeding
Thrombosis
Plentiful, large, abnormal platelets on peripheral smear
Erythromelalgia

A

ESSENTIAL THROMBOCYTHEMIA

Massive proliferation of megakaryocytes and platelets

Note - May be JAK2+

114
Q

Massive splenomegaly
Hepatomegaly
Teardrop RBCs
Dry tap

A

PRIMARY MYELOFIBROSIS

Obliteration of bone marrow with fibrosis due to increased fibroblast activity

Note - May be JAK2+

115
Q

Increased RBC mass
Decreased O2 sat
Increased EPO

A

APPROPRIATE ABSOLUTE POLYCYTHEMIA

Due to lung disease, CHF, or high altitude

Note - Malignancy and Hydronephrosis present with inappropriate absolute polycythemia (normal O2 sat, ectopic EPO)

116
Q

Mechanism of heparin induced thrombosis and thrombocytopenia

A

HIT

Development of IgG antibodies against bound platelet factor 4 (PF4) - antibody-heparin-PF4 complex activates platelets

117
Q

Mutation affecting metabolism of Warfarin

A

Polymorphism in vitamin K epoxide reductase complex (VKORC1)

118
Q

Mechanism of Warfarin induced skin necrosis

A

Protein C/S affected early leading to transient early microthrombosis - prevent with Heparin bridge

119
Q

Failure of PT to correct with Vit K supplementation

A

LIVER FAILURE

Factor VII is affected first

Note - Factor VIII is only factor made by endothelial cells rather than the liver (abnormal aPTT, normal PT)

120
Q

O2 content, SaO2, PaO2 in…

CO poisoning
CN poisoning
Anemia (reduced Hgb)
Polycythemia (increased Hgb)
High altitude

Note - O2 content = Hgb*SaO2 + PaO2

A 
Decreased, Decreased, Normal
Normal, Normal, Normal
Decreased, Normal, Normal
Increased, Normal, Normal
Decreased, Decreased, Decreased
121
Q

Mechanism of uremic bleeding - Increased BT

A

Qualitative platelet dysfunction due to uremic toxins - PT, aPTT, and platelet count should be normal

122
Q

Causes of Pure Red Cell Aplasia (PRCA) - Severe hypoplasia of marrow erythroids with normal granulopoiesis and thrombopoiesis

A

Thymoma
ALL
B19

123
Q

Electrolytes in tumor lysis syndrome

A

Hyperkalemia
Hyperuricemia
Hyperphosphatemia
Hypocalcemia

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