Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Step 1 > Hematology and Oncology > Flashcards

Hematology and Oncology Flashcards

1
Q

RBC lacks

A

Nucleus and organelles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

RBC source of energy

A

Glucose → 90% used in glycolysis, 10% used in HMP shunt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Compare anisocytosis and poikilocytosis

A

Anisocytosis → varying sizes

Poikilocytosis → varying shapes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Granules stored in platelets

A

Dense granules → ADP, Ca2+

Alpha granules → vWF, fibrinogen, fibronectin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Granulocytes

A

Neutrophils, eosinophils, basophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Mononuclear cells

A

Monocytes, lymphocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Granules stored in neutrophils

A

Specific granules → leukocyte alkaline phosphatase (LAP), collagenase, lysozyme, lactoferrin

Azurophilic granules → lysosomes, proteinases, acid phosphatase, myeloperoxidase, β-glucuronidase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Neutrophilic chemotactic agents

A
  • C5a
  • IL-8
  • LTB4
  • Kallikrein
  • Platelet-activating factor
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Macrophages are an important component in the formation of what

A

Granulomas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Lipid A from bacterial LPS binds what to initiate septic shock

A

CD14 on macrophages

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Causes of eosinophilia

A
  • Neoplasia
  • Asthma
  • Allergic processes
  • Chronic adrenal insufficiency
  • Parasites (invasive)

“NAACP”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Eosinophils produce

A
  • Histaminase

- Major basic protein (a helminthotoxin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Granules in basophils contain

A
  • Heparin (anticoagulant)
  • Histamine (vasodilatory)
  • Leukotrienes are synthesized and released on demand
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Degranulation of mast cells leads to release of

A
  • Histamine
  • Heparin
  • Tryptase
  • Eosinophil chemotactic factors

Mast cells contain basophilic granules, just like basophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What cells originate from the same precursor as basophils

A

Mast cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Where are plasma cells found

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Where does fetal erythropoiesis occur

A
  • Yolk sac (3-8 weeks)
  • Liver (6 weeks - birth)
  • Spleen (10-28 weeks)
  • Bone marrow (18 weeks to adult)

“Young Liver Synthesizes Blood”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Migration pattern for hemoglobin

A

Furthest from cathode (-)

Normal adult (AA) → Normal newborn (AF) → Sickle cell trait (AS) → Sickle cell disease (SS) → HbC train (AC) → Hb C disease (CC) → Hb SC disease (SC)

“A Fat Santa Claus”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Anticoagulant with greatest efficacy against factors Xa and IIa

A

Factor Xa → LMWH

Factor IIa (thrombin) → heparin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Enzyme responsible for reducing vitamin K

A

Epoxide reductase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

vWF protects and carries which factor

A

VIII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Antithrombin inhibits activated forms of which factors

A

II, VII, IX, X, XI, XII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the principal targets of antithrombin

A

Thrombin and factor Xa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How do protein C and S work as anticoagulants

A

Protein C → Activated Protein C
- via Thrombin-thrombomodulin complex in endothelial cells

Activated Protein C + Protein S → Cleaves and inactivates Va, VIIIa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Describe primary hemostasis

A

INJURY→ endothelial damage → transient vasoconstriction via neural stimulation reflex and endothelin (released from damaged cell)

EXPOSURE → vWF binds to exposed collagen; vWF is from Weibel-Palade bodies of endothelial cells and α - granules of platelets

ADHESION → platelets bind vWF via GpIb receptor at the site of injury only (specific) → platelets undergo conformational changes → platelets release ADP and Ca2+ (necessary for coagulation cascade) and TXA2 → ADP helps platelets adhere to endothelium

ACTIVATION → ADP binding to receptor induces GpIIb/IIIa expression at platelet surface

AGGREGATION → fibrinogen binds GpIIb/IIIa receptors and links platelets

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Pro-aggregation factors

A
  • TXA2 (released by platelets)
  • ↓ blood flow
  • ↑ platelet aggregation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Anti-aggregation factors

A
  • PGI2 and NO (released by endothelial cells)
  • ↑ blood flow
  • ↓ platelet aggregation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Mechanism of ristocetin

A
  • Activates vWF to bind GpIb

- Failure of agglutination with ristocetin assay occurs in von Willebrand disease and Bernard-Soulier syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Acanthocyte

A
  • Liver disease

- Abetalipoproteinemia (states of cholesterol dysregulation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Basophilic stipiling

A
  • Lead poisoning
  • Sideroblastic anemias
  • Myelodysplastic syndromes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Dacrocyte

A

Bone marrow infiltration (eg myelofibrosis, osteopetrosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Degmacyte

A

G6PD deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Echinocyte

A
  • End-stage renal disease
  • Liver disease
  • Pyruvate kinase deficiency

Different from acanthocyte; its projections are more uniform and smaller

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Elliptocyte

A
  • Hereditary elliptocytosis (usually asymptomatic)

- Caused by mutation in genes encoding RBC membrane proteins (eg spectrin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Macro-ovalocyte

A
  • Megaloblastic anemia (also, hypersegmented PMNs)

- Marrow failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Ringed sideroblast

A
  • Sideroblastic anemia

- Excess iron in mitochondria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Schistocyte

A
  • DIC
  • TTP/HUS
  • Mechanical hemolysis (eg heart valve prosthesis)

Examples include “helmet cells”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Sickle cell

A

Sickle cell anemia

Sickling occurs with dehydration, deoxygenation and at high altitude

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Spherocyte

A
  • Hereditary spherocytosis

- Drug and infection induced hemolytic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Target cell

A
  • HbC
  • Asplenia
  • Liver disease
  • Thalassemia

“HALT”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Heinz bodies

A

G6PD deficiency

Oxidation of Hb-SH groups to -S-S- → Hb precipitation (Heinz bodies) with subsequent phagocytic damage to RBC membrane → degmacyte (“bite cell”)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Howell-Jolly bodies

A
  • Seen in patients with functional hyposplenia or asplenia
  • Basophlic nuclear remnants found in RBCs
  • Howell-Jolly bodies are normally removed from RBCs by splenic macrophages
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

How does lead poisoning cause basophilic stippling

A

Inhibits rRNA degradation → RBCs retain aggregates of rRNA (basophilic stippling)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Symptoms of lead poisoning

A
  • Lead Lines on gingivae (Burton lines) and on metaphyses of long bones on x-ray
  • Encephalopathy and Erythrocyte basophilic stippling
  • Abdominal colic and sideroblastic Anemia
  • Drops - wrist and foot drop

“LEAD”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Treatment of lead poisoning

A
  • Dimercaprol and EDTA are 1st line of treatment

- Succimer used for chelation for kids (“it SUCks to be a kid who eats lead”)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Causes of sideroblastic anemia

A
  • Defect in heme synthesis due to X linked defect in ALA synthase gene
  • CAUSES: genetic, acquired (myelodysplastic syndromes), and reversible (alcohol is most common, also lead, B6 deficiency, copper deficiency, isoniazid)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Treatment of sideroblastic anemia

A

Pyridoxine (B6, cofactor for ALA synthase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Orotic aciduria

A
  • Inability to convert orotic acid → UMP (de novo pyrimidine synthesis pathyway) because of defect in UMP synthase
  • AR
  • Present in children as failure to thrive, developmental delay and megaloblastic anemia refractory to folate and B12
  • NO hyperammonemia (vs ornithine transcarbamylase deficiency → ↑ orotic acid with hyperammonemia)
  • Orotic acid found in urine
  • TREATMENT: UMP to bypass mutated enzyme
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Diamond-Blackfan anemia

A
  • Rapid-onset anemia within 1st year of life due to intrinsic defect in erythroid progenitor cells
  • ↑ % HbF (but ↓ total Hb)
  • Presents with short stature, craniofacial abnormalities, and upper extremity malformations (triphalangeal thumbs)
50
Q

Nonmegaloblastic macrocytic anemia

A
  • Macrocytic anemia in which DNA synthesis is unimpaired
  • RBC macrocytosis without hypersegmented neutrophils
  • Causes include alcoholism and liver disease
51
Q

Intravascular hemolysis findings

A
  • ↓ haptoglobin
  • ↑ LDH
  • Schistocytes and ↑ reticulocytes on blood smear
  • Characteristic hemoglobinuria, hemosiderinuria, and urobilinogen in urine
  • May also seen ↑ unconjugated bilirubin
  • Causes include mechanical hemolysis (eg prosthetic valve), paroxysmal nocturnal hemoglobinuria, microangiopathic hemolytic anemias
52
Q

Extravascular hemolysis findings

A
  • Macrophages in sleep clear RBCs
  • Spherocytes in peripheral smear
  • ↑ LDH
  • NO hemoglobinuria/hemosiderinuria
  • ↑ unconjugated bilirubin (can cause jaundice)
  • Can present with urobilinogen in urine
53
Q

Pathogenesis of anemia of chronic disease

A

Inflammation → ↑ hepcidin (released by liver, binds ferroportin on intestinal mucosal cells and macrophages, thus inhibiting iron tranport) → ↓ release of iron from macrophages and ↓ iron absorption from gut

54
Q

Treatment of anemia of chronic disease in the case of chronic kidney disease

A

Erythropoietin

55
Q

Causes of aplastic anemia

A

Caused by failure or destruction of myeloid stem cells due to:

  • Radiation and drugs → benzene, chloramphenicol, alkylating agents, antimetabolites
  • Viral agents → parvovirus B19, EBV, HIV, hepatitis viruses
  • Fanconi anemia → DNA repair defect with NHEJ causing bone marrow failure as well as short stature, ↑ incidence of tumors/leukemias, cafe-au-lait spots, thumb/radial defects
  • Idiopathic (immune mediated, primary stem cell defect), may follow acute hepatitis
56
Q

Presentation of hereditary spherocytosis

A
  • Splenomegaly

- Aplastic crisis (parvovirus B19 infection)

57
Q

Hereditary spherocytosis - intravascular or extravascular hemolysis

A

Extravascular hemolysis

58
Q

Presentation of G6PD deficiency

A
  • Back pain

- Hemoglobinuria a few days after oxidant stress

59
Q

Inheritance pattern of G6PD deficiency

A

X linked recessive

60
Q

G6PD deficiency - intavascular or extravascular hemolysis

A

Both

61
Q

Pyruvate kinase deficiency

A
  • AR
  • Defect in pyruvate kinase → ↓ ATP → rigid RBCs → extravascular hemolysis
  • Hemolytic anemia in a newborn
62
Q

Hb C disease - intravascular or extravascular hemolysis

A

Extravascular

63
Q

What does the blood smear show for Hb C disease homozygotes

A

Blood smear shows hemoglobin crystals inside RBCs and target cells

64
Q

Triad of paroxysmal nocturnal hemoglobinuria

A
  • Coombs (-) hemolytic anemia
  • Pancytopenia
  • Venous thrombosis
65
Q

Cause of paroxysmal nocturnal hemoglobinuria

A

Acquired mutation in a hematopoietic stem cell. ↑ incidence of acute leukemias.

66
Q

Paroxysmal nocturnal hemoglobinuria - intravascular or extravascular hemolysis

A

Intravascular

67
Q

Treatment of paroxysmal nocturnal hemoglobinuria

A

Eculizumab → terminal complement inhibitor

68
Q

Sickle cell anemia - intravascular or extravascular hemolysis

A

Both

69
Q

Treatment of sickle cell anemia

A

Hydroxyurea and hydration

70
Q

Compare microangiopathic and macroangiopathic anemia

A

Microangiopathic → RBCs are damaged when passing through obstructed or narrowed vessel lumina; seen in DIC, TTP/HUS, SLE, and malignant hypertension

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

Schistocytes are seen on peripheral blood smear for both

71
Q

Iron deficiency anemia lab values

A
  • ↓ serum iron (primary)
  • ↑ TIBC
  • ↓ ferritin
  • ↓ % transferrin saturation
72
Q

Chronic disease lab values

A
  • ↓ serum iron
  • ↓ TIBC
  • ↑ ferritin (primary)
73
Q

Hemochromatosis lab values

A
  • ↑ serum iron (primary)
  • ↓ TIBC
  • ↑ ferritin
  • ↑↑ % transferrin saturation
74
Q

Pregnancy/OCP use

A
  • ↑ TIBC (primary)

- ↓ % transferrin saturaiton

75
Q

Why do corticosteroids cause neutrophilia despite causing eosinopenia and lymphopenia

A

Corticosteroids ↓ activation of neutrophil adhesion molecules, impairing migration out of the vasculature to sites of inflammation. In contrast, corticosteroids sequester eosinophils in lymph nodes and cause apoptosis of lymphocytes

76
Q

Causes of neutropenia

A
  • Sepsis/post-infection
  • Drugs (including chemotherapy)
  • Aplastic anemia
  • SLE
  • Radiation
77
Q

Causes of lymphopenia

A
  • HIV
  • DiGeorge syndrome
  • SCID
  • SLE
  • Corticosteroids
  • Radiation
  • Sepsis
  • Postoperative
78
Q

Causes of eosinopenia

A
  • Cushing syndrome

- Corticosteroids

79
Q

Left shift

A
  • ↑ neutrophil precursors, such as band cells and metamyelocytes, in peripheral blood
  • Usually seen with neutrophilia in the acute response to infection or inflammation
  • Called LEUKOERYTHROBLASTIC REACTION when left shift is seen with immature RBCs → occurs with severe anemia (physiologic response) or marrow response (eg fibrosis, tumor taking up space in marrow)
80
Q

Lead poisoning

A
  • Affected enzymes → ferrochelatase, ALA dehydratase
  • Accumulated substrates → protoporphyrin, ALA (blood)

Presentation:

  • Microcytic anemia (basophilic stippling), GI, kidney disease
  • Children → exposure to lead paint → mental deterioration
  • Adults → environmental exposure (eg batteries, ammunition) → headache, memory loss, demyelination
81
Q

Acute intermittent porphyria

A
  • Affected enzyme → porphobilinogen deaminase
  • Accumulated substrates → porphobilinogen, ALA, corphobilinogen (urine)

Presentation (5 Ps):

  • Painful abdomen
  • Port wine-colored urine
  • Polyneuropathy
  • Psychological disturbances
  • Precipitated by drugs (eg cytochrome P450 inducers), alcohol, starvation

Treatment → glucose and heme, which inhibit ALA synthase

82
Q

Porphyria cutanea tarda

A
  • Affected enzyme → uroporphyrinogen decarboxylase
  • Accumulated substrate → uroporphyrin (tea-colored urine)

Presentation:

  • Blistering cutaneous photosensitivity
  • Most common porphyria
83
Q

Iron poisoning

A
  • Cell death due to peroxidation of membrane lipids
  • Symptoms → N/V, gastric bleeding, lethargy, scarring leading to GI obstruction
  • Treatment → chelation and dialysis → IV deferoxamine, oral deferasirox
84
Q

PT tests function of what

A
  • Common and extrinsic pathway

- Factors I, II, V, VII, and X

85
Q

PTT tests function of what

A
  • Common and intrinsic pathway

- All factors except VII and XIII

86
Q

PT and PTT in vitamin K deficiency

A

↑ PT, ↑ PTT

87
Q

Bernard-Soulier syndrome

A
  • -/↓ platelet count
  • ↑ BT
  • Defect in platelet plug formation
  • ↓ GpIb → defect in platelet-to-vWF adhesion
  • Large platelets
88
Q

Glanzmann thrombasthenia

A
    • platelet count
  • ↑ BT
  • Defect in platelet plug formation
  • ↓ GpIIb/IIIa → defect in platelet-to-platelet aggregation
  • Labs: blood smear shows no platelet clumping
89
Q

Hemolytic-uremic syndrome

A
  • ↓ platelet count
  • ↑ BT
  • Characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure
  • Typical HUS seen in children, accompanied by diarrhea and commonly caused by Shiga-toxin producing E coli (STEC)
  • HUS in adults does NOT present with diarrhea, STEC infection is NOT required
  • Same spectrum as TTP, with a similar clinical presentation and same initial treatment of plasmapheresis
90
Q

Immune thrombocytopenia

A
  • ↓ platelet count
  • ↑ BT
  • Anti-GpIIb/IIIa antibodies → splenic macrophage consumption of platelet-antibody complex
  • Commonly due to viral illness
  • Labs: ↑ megakaryocytes on bone marrow biopsy
  • Treatment: steroids, IVIG, splenectomy for refractory ITP
91
Q

Thrombotic thombocytopenic purpura

A
  • ↓ platelet count
  • ↑ BT
  • Inhibition or deficiency of ADAMTS 13 (vWF metalloprotease) → ↓ degradation of vWF multimers
  • Pathogenesis: ↑ large vWF multimers → ↑ platelet adehesion → ↑ platelet aggregation and thrombosis
  • Labs: schistocytes, ↑ LDH
  • Symptoms: pentad of neurologic and renal symptoms, fever, thrombocytopenia, and microangiopathic hemolytic anemia
  • Treatment: plasmapheresis, steroids
92
Q

von Willebrand disease

A
    • platelet count
  • ↑ BT
    • PT
  • -/↑ PTT
  • Intrinsic pathway coagulation defect: ↓ vWF → ↑ PTT (vWF acts to carry/ protect factor VIII)
  • Defect in platelet plug formation: ↓ vWF → defect in platelet-to-vWF adhesion
  • AD
  • Mild but most common inherited bleeding disorder
  • No platelet aggregation with ristocetin cofactor assay
  • Treatment: desmopressin (releases vWF stored in endothelium)
93
Q

Disseminated intravascular coagulation

A
  • ↓ platelet count
  • ↑ BT
  • ↑ PT
  • ↑ PTT
  • Widespread activation of clotting → deficiency in clotting factors → bleeding state
  • Causes: Sepsis (gram -), Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion (“STOP Making New Thrombi”)
  • Labs: schistocytes, ↑ fibrin degradation products (D dimers), ↓ fibrinogen, ↓ factors V and VIII
94
Q

Antithrombin deficiency

A
  • Inherited deficiency of antithrombin → has no direct effect on the PT, PTT or thrombin time but diminitshes the increase in PTT following heparin administration
  • Can also be acquired: renal failure/ nephrotic syndrome → antithrombin loss in urine → ↓ inhibition of factors IIa and Xa
95
Q

Factor V Leiden

A
  • Production of mutant factor V (G → A DNA point mutation → Arg506Gln mutation near the cleavage site) that is resistant to degradation by activated protein C
  • Most common cause of inherited hypercoagulability in Caucasians
  • Complications include DVT, cerebral vein thromboses, recurrent pregnancy loss
96
Q

Protein C or S deficiency

A
  • ↓ ability to inactivate factor Va and VIIIa
  • ↑ risk of thrombotic skin necrosis with hemorrhage after administration of warfarin
  • If this occurs, think protein C deficiency
  • “Together protein C Cancels and protein S Stops Coagulation”
97
Q

Prothrombin gene mutation

A

Mutation in 3’ untranslated region → ↑ production of prothrombin → ↑ plasma levels and venous clots

98
Q

Packed RBCs

A
  • ↑ Hb and O2 carrying capacity

- Clinical use → acute blood loss, severe anemia

99
Q

Platelets

A
  • ↑ platelet count (↑ by approximately 5000/mm3/unit)

- Clinical use → stop significant bleeding (thrombocytopenia, qualitative platelet defects)

100
Q

Fresh frozen plasma

A
  • ↑ coagulation factor levels

- Clinical use → DIC, cirrhosis, immediate warfarin reversal

101
Q

Cryoprecipitate

A
  • Contains fibrinogen, factor VIII, factor XIII, vWF, and fibronectin
  • Clinical use → coagulation factor deficiencies involving fibrinogen and factor VIII
102
Q

Risks of blood transfusions

A
  • Infection transmission (low)
  • Transfusion reactions
  • Iron overload (may lead to secondary hemochromatosis)
  • Hypocalcemia (citrate is a Ca2+ chelator)
  • Hyperkalemia (RBCs may lyse in old blood units)
103
Q

Compare Hodgkin and Non-Hodgkin lymphomas

A

Hodgkin:

  • Localized, single group of nodes
  • Contiguous spread (stage is strongest predictor of prognosis)
  • Many patients have a relatively good prognosis
  • Characterized by Reed-Sternberg cells
  • Bimodal distribution → young adulthood and >55 years; more common in men except for nodular sclerosing type
  • Associated with EBV
  • Constitutional (“B”) signs/symptoms”: low grade fever, night sweats, weight loss

Non-Hodgkin:

  • Multiple lymph nodes involved
  • Extranodal involvement common
  • Noncontiguous spread
  • Majority involve B cells; a few are of T cell lineage
  • Can occur in children and adults
  • May be associated with HIV and autoimmune diseases
  • May present with constitutional signs/symptoms
104
Q

Primary central nervous system lymphoma

A
  • Non-Hodgkin lymphoma
  • Neoplasm of mature B cells
  • Occurs in adults
  • Most commonly associated with HIV/AIDS
  • Considered an AIDS-defining illness
  • Variable presentation: confusion, memory loss, seizures
  • Mass lesion(s) on MRI, needs to be distinguished from toxoplasmosis via CSF analysis or other lab tests
105
Q

Waldenstrom macroglobulinemia

A
  • M spike = IgM
  • Hyperviscosity syndrome (eg blurred vision, Raynaud phenomenon)
  • No CRAB findings
106
Q

Monoclonal gammopathy of undetermined significance (MGUS)

A
  • Monoclonal expansion of plasma cells
  • Asymptomatic
  • May lead to multiple myeloma
  • No CRAB findings
  • Patients with MGUS develop multiple myeloma at a rate of 1-2% per year
107
Q

Myelodysplastic syndromes

A
  • Stem-cell disorders involving ineffective hematopoiesis → defects in cell maturation of all nonlymphoid lineages
  • Caused by de novo mutation or environmental exposures (eg radiation, benzene, chemotherapy)
  • Risk of transformation to AML
108
Q

Pseudo-Pelger-Huet anomaly

A
  • Neutrophils with bilobed nuclei
  • Typically seen after chemotherapy
  • Myelodysplastic syndrome
109
Q

Lukemia cutis

A

Lukemic cell infiltration of skin

110
Q

Rare leukemias present with

A

Normal/ ↓ WBCs

111
Q

Lymphoid neoplasm associated with autoimmune hemolytic anemia

A

Chronic lymphocytic leukemia/ small lymphocytic lymphoma

112
Q

Richter transformation

A

SLL/CLL transformation into an aggressive lymphoma, most commonly diffuse large B cell lymphoma

113
Q

Patients with hairy cell leukemia generally present with

A

Massive splenomegaly

114
Q

Treatment of hairy cell leukemia

A
  • Cladribine

- Pentostatin

115
Q

Are dendritic/Langerhans cells of Langerhans cell histiocytosis functional

A

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

116
Q

Presentation of polycythemia vera

A
  • Erythromelalgia → severe, burning pain and red-blue coloration due to episodic blood clots in vessels of the extremities
  • Itching after hot shower
  • These symptoms respond to aspirin
117
Q

Relative polycythemia

A
  • ↓ plasma volume
    • RBC mass
    • O2 saturation
    • EPO
  • Associated with dehydration and burns
118
Q

Appropriate absolute polycythemia

A
    • plasma volume
  • ↑ RBC mass
  • ↓ O2 saturation
  • ↑ EPO levels
  • Associated with lung disease, congenital heat disease, and high altitude
119
Q

Inappropriate absolute polycythemia

A
    • plasma volume
  • ↑ RBC mass
    • O2 saturation
  • ↑ EPO
  • Associated with malignancy (eg renal cell carcinoma, hepatocellular carcinoma), hydronephrosis
  • Due to ectopic EPO secretion
120
Q

Polycythemia vera

A
  • ↑ plasma volume
  • ↑↑ RBC mass
    • O2 saturation
  • ↓ EPO (due to negative feedback)

Step 1 (25 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions