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Hematology 3% Flashcards

1
Q

Multiple myeloma shows ____ RBCs.

A

Rouleaux formation d/t increase plasma proteins

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2
Q

B12 and Folate deficiency presents as (macro/micro)cytic anemia and ____.

A

MACROcytic anemia

hypersegmented neutrophils

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3
Q

Heinz bodies =

Seen in ____

A

denatured Hgb in cells

Thalassemias (alpha thalassemia intermedia)
G6PD Deficiency

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4
Q

Howell-Jolly Bodies seen in ____.

A

Splenectomized pts

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5
Q

Target cells seen in ___.

A

Sickle cell disease
Thalassemia
Hgb SC

Severe Fe deficiency
Asplenia
Liver disease

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6
Q

Echinocytes (a.k.a. ____) seen in ____ (3)

A

Burr cells

Uremia *
Pyruvate kinase deficiency
Hypophosphatemia

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7
Q

Acanthocytes (a.k.a. ___) seen in ____ (5)

A

Spur cells

Liver disease (ETOHic cirrhosis) 
Post splenectomy
Thalaseemia
Autoimmune Hemolytic Anemia
Renal Disease
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8
Q

Reticulocytosis =

Seen when ___

A

Immature RBC

Increased blood loss
Increased RBC destruction (hemolytic anemia)

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9
Q

Fe deficiency anemia
(increase/decrease) serum Fe
(increase/decrease) ferritin
(increase/decrease) TIBC

A

decrease serum Fe
decrease ferritin
INCREASE TIBC

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10
Q 
Anemia of Chronic Disease
(increase/decrease) serum Fe
(increase/decrease) ferritin
(increase/decrease) TIBC
(increase/decrease) Haptoglobin
(increase/decrease) CRP
(increase/decrease) Hepcidin
A
  • decrease serum Fe
  • INCREASE ferritin
  • DECREASE TIBC
  • Increase Haptoglobin = binds free hemoglobin reducing iron availability for microbes
  • Increase CRP
  • Increase Hepcidin = prevent iron release from macrophages –> reducing iron availability for microbes
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11
Q

Low reticulocytes seen in ____

A

Fe, B12, Folate deficiencies

Anemia of chronic disease

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12
Q

High reticulocytes seen in ____

A

Blood loss

Hemolytic anemias

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13
Q

Indirect hyperbilirubinemia is associated with ___.

Direct hyperbilirubinemia is associated with ___.

A

Indirect: Jaundice

Direct: dark urine b/c water soluable

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14
Q 
Hemolytic anemia:
(increase/decrease) reticulocytes
(increase/decrease) LDH
(increase/decrease) indirect bilirubin
(increase/decrease) haptoglobin
A

Increase reticulocytes
Increase LDH = enzyme in RBCs –> RBC destruction –> increase LDH
Increase indirect bilirubin
DECREASE Haptoglobin = increase RBC destruction –> increased free Hgb –> haptoglobin binds to free Hgb –> haptoglobin stores used up

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15
Q

Alpha Thalassemia:

Hgb ratio

A

Normal Hgb ratios of HgbA, A2 and F

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16
Q

Beta Thalassemia:

Hgb ratio

A

Decreased HgbA
Increased HgbA2
Increased HbgF

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17
Q

T/F: TTP and HUS can be distinguished via abnormal coag labs (prolonged PT and PTT).

A

False. Both have normal coags, can’t be distinguished via labs.

Prolonged PT and PTT seen in Disseminated Intravascular Coagulation (DIC)

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18
Q

TTP presentation

A 
Thrombocytopenia
Hemolytic anemia
Kidney damage 
Neuro sx**
Fevers**
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19
Q

HUS presentation

A

Thrombocytopenia
Hemolytic anemia
Kidney damage

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20
Q

“Dark urine worse in AM”

A

Paroxysmal nocturnal hemoglobinuria

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21
Q

Schilling test used to diagnose ____.

Other findings (3)

A

Pernicious Anemia (B12 deficiency)

+ Intrinsic factor ab
Parietal cell ab
Increased gastrin levels

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22
Q

B12 is absorbed in ____

Folate is absorbed in ___

A

B12: ileum
Folate: jejunum

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23
Q

Watch for signs of ____ when giving B12 replacement

A

HYPOkalemia

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24
Q

T/F: Folate deficiency causes pallor, glossitis, stomatitis and neurologic sx (peripheral neuropathy, spinal cord demyelination** and degeneration)

A

False. B12 causes neurological symptoms, NOT folate. Otherwise, both present similarly.

Neurologic sx of B12 deficiency: peripheral neuropathy, ataxia, weakness, vibratory, sensory and proprioception deficits, DECREASED DTR

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25
T/F: Replacing folate in pts w/ B12 deficiency will correct anemia but worsen neuro sx.
True
26
Microcytic anemia w/ normal/increased Fe or no response to Fe tx =
Thalassemia
27
HgbA = HgbA2 = HgbF =
HgbA = aabb HgbA2 = aadd HgbF =aagg ``` a= alpha b= beta d= delta g = gamma ```
28
What type of thalassemia? 1. normal Hgb ratio 2. Increased HgbA2, increased HgbF, decreased HgbA 3. Increased HgbA2, increased HgbF (90%), little or no HgbA
1. Alpha thalassemia (mior, intermedia, major) 2. Beta thalassemia minor 3. Beta thalassemia major (Cooley's)
29
Hgb Barts seen in ___
alpha thalassemia major (Hydrops fetalis) =gamma tetramers Associated w/ stillbirth, or death soon after
30
Deferoxamine
Iron chelating agents | Tx of severe anemia, Apha/beta thalassemia major
31
Pts with beta-thalassemia major will become symptomatic at age ___.
6 months (when HgbF declines)
32
Plummer Vinson syndrome =
Dysphagia + esophageal webs + atrophic glossitis + Fe deficiency
33
Anemia of chronic disease: (macro/normo/micro)chromic (macro/normo/micro)cytic anemia Tx:
Normochromic, normocytic anemia Tx: Erythropoietin-alpha
34
``` G6PD Deficiency: Peripheral smear shows ____ Labs: (increased/decreased) reticulocytes (increased/decreased) (indirect/direct) bilirubin (increased/decreased) haptoglobin ```
Schistocytes Heinz bodies Increased reticulocytes Increased indirect bilirubin Decreased haptoglobin
35
Sickle cell disease dx: Hemoglobin electrophoresis Peripheral smear
Increased HgbF, HgbS, NO HgbA Target cells, sickled erythrocytes, Howell-Jolly bodies (functional asplenia)
36
Sickle cell disease tx: Pain control Severe pain crisis Immunization against ___
Pain control: IV hydration and oxygen 1st step in pain crisis Hydroxyurea: SEVERE pain crisis Immunize against: S. pneumococcus, H. Influenza B, N. Meningococcus (SHiN)
37
Do NOT give ____ in Sickle Cell Disease pain crisis.
Meperidine (Demerol) | May lead to seizures and renal failure
38
Signs of Sickle cell disease present at age ____. MC 1st presentation = Aplastic crisis associated w/ ___. Osteomyelitis MC caused by ___.
6 months Dactylitis Aplastic crisis: Parvovirus B-12 Osteomyelitis: Salmonella
39
PTT measures ___ pathway. Involves ___ factors.
intrinsic and common pathway | Factors 1, 2, 5, 8**, 9**, 10, 11**, 12**
40
Warfarin therapy causes prolonged (PT/PTT) | Heparin therapy causes prolonged (PT/PTT)
Warfarin: PT Heparin: PTT
41
PT measures ___ pathway. Involves ___ factors.
Extrinsic and common pathway | Factors 1, 2, 5, 7**, 10**
42
DIC causes prolonged (PT/PTT) Vit K deficiency causes prolonged (PT/PTT) vWD causes prolonged (PT/PTT) Hemophilia A/B causes prolonged (PT/PTT)
DIC causes prolonged PT AND PTT Vit K deficiency causes prolonged PT vWD causes prolonged PTT Hemophilia A/B causes prolonged PTT
43
Heparin overdose antidote = | Warfarin overdose antidote =
Heparin OD = Protamine sulfate Warfarin OD = Vitamin K
44
Antibody against ADAMTS13 seen in ____. PT/PTT: Tx:
Thrombotic Thrombocytopenic Purpura (TTP) Normal PT/PTT Tx: Plasmapheresis *** Immunosuppression: steroids, cyclophosphamides
45
Difference between TTP and HUS presentation
Kidney failure more common in HUS | Fever and neurologic sx (HA, CVA, AMS) in TTP, NOT HUS
46
Suspect ___ if renal failure in children with diarrhea prodrome
Hemolytic Uremic Syndrome HUS usually preceded by Enterohemorrhagic E. Coli 0157:H&, Shigella, or Salmonella gastroenteritis
47
Platelet activation by exotoxins seen in ___. | PT/PTT:
Hemolytic Uremic Syndrome | Normal PT/PTT
48
T/F: Antibiotics are useful in treatment of HUS.
False. May actual worsen condition d/t increased verotoxin release w/ cell lysis.
49
``` Disseminated Intravascular Coagulation (DIC) (increase/decrease) PTT (increase/decrease) PT (increase/decrease) INR (increase/decrease) fibrinogen ```
Increase PTT/PT/INR Decrease fibrinogen Also increased D-dimer
50
Tx of Disseminated Intravascular Coagulation
Fresh Frozen Plasma: if severe bleeding Cryoprecipiate: replace fibrinogen Platelet transfusion: ONLY if <20K
51
T/F: Idiopathic (Autoimmune) Thromobocytopenic Purpura presents with mucocutaneous bleeding (purpura, bruises, petechiae, bullae) and splenomegaly.
False. NO splenomegaly
52
Acute ITP seen in MC___
children/boys after viral infection --> self limited
53
Tx of ITP
Children: observation +/- IVIG Adults: Corticosteroids --> IVIG --> splenectomy if refractory
54
T/F: Petechiae is commonly seen in Hemophilia A/B.
False. Not common in Hemophilia A/B. Common in Von Willebrand Disease.
55
Von Willebrand Disease: Bleeding time and PTT prolongation worsen with ___. Gold standard dx:
worsen with aspirin Decreased Ristocetin activity test: No platelet aggregation w/ Ristocetin
56
Von Willebrand Disease: Prolonged (PTT/PT) (Does/does not) correct w/ mixing study.
PTT | DOES correct
57
Tx of Type I Von Willebrand Disease Mild: Moderate: Severe:
Mild: NO treatment Moderate: DDAVP Severe: Cryoprecipitate
58
Reed sternberg cells is pathognomonic for ____.
Hodgkin's Lymphoma
59
T/F: Hodgkin lymphoma is more difficult to cure than Non-Hodgkin lymphoma.
False. Hodgkin lymphoma is highly curable compared to NHL.
60
Hodgkin or Non-Hodgkin lymphoma: 1. Upper body lymph nodes 2. MC in >50 y/o 3. Associated w/ clonal B-cell proliferation 4. Extra nodal sites are common
1. Upper body lymph nodes: HL Peripheral lymph nodes: NHL 2. MC in >50 y/o: NHL Bimodal 20 y/o and > 50 y/o: HL 3. Associated w/ clonal B-cell proliferation: HL 4. Extra nodal sites are common: NHL Pel-Ebstein fevers: HL
61
MC indolent Non-hodgkin lymphoma
Follicular: Not curable generally
62
MC aggressive Non-hodgkin lymphoma
Diffuse Large B cell: curable w/ chemo
63
Clinical manifestation of Multiple Myeloma
``` "BREAK" Bone pain Recurrent infections Elevated calcium Anemia Kidney Failure ```
64
Increased risk of multiple myeloma seen in ___
>65 y/o African Americans men
65
Single clone of plasma cells, accumulate in bone marrow interrupting marrow's normal cell production.
Multiple myeloma Increased monoclonal Ab (esp IgG, IgA)
66
Dx of Multiple Myeloma
Serum Protein Electrophoresis: monoclonal protein spike Urine Protein Electrophoresis: Bence-Jones proteins Rouleaux formation, Increased ESR Skull xray: Punched out lesions Bone scan NOT helpful
67
Bence Jones proteins seen in ___
Multiple Myeloma
68
Philadelphia Chromosome = translocation between chromosome ___, causing fusion gene ___. Seen in ___.
9 and 22 gene bcr-abl CML
69
MC childhood leukemia malignancy Bone marrow shows:
ALL Hypercellular > 20% blasts
70
Auer rods seen in ___
AML
71
MC form of leukemia in adults: | MC acute leukemia in adults:
MC form of leukemia in adults: CLL MC acute leukemia in adults: AML
72
Tumor lysis syndrome caused by ___. | Management:
chemotx initiation d/t large number of cells being destroyed - Hyperuricemia, HyperK, HypoCa, HyperPhosphate, acute renal failure Management: Allopurinol
73
Acquired myeloproliferative d/o w/ overproduction of all 3 stem cell lines.
Polycythemia Vera
74
Polycythemia Vera: (increase/decrease) Hematocrit/RBC (increase/decrease) erythropoietin levels (increase/decrease) WBCs (increase/decrease) Leukocyte alkaline phosphatase (increase/decrease) B-12
``` Increased Hematocrit/RBC Decreased erythropoietin levels Increased WBCs Increased Leukocyte alkaline phosphatase Increased B-12 ```
75
Polycythemia Vera tx
Phlebotomy** | Hydroxyurea
76
Primary erythrocytosis is distinguished from secondary erythrocytosis by ____.
normal WBC/platelets in 2ry Secondary d/t: Hypoxia (ex COPD) Pathologic (renal dz) Reactive polycythemia (normal RBCs mass in setting of decreased plasma volume)
77
C282Y HFE genotype seen in ____
hereditary hemochromatosis
78
``` Hereditary hemochromatosis (increase/decrease) serum iron (increase/decrease) serum transferrin saturation (increase/decrease) ferritin (increase/decrease) TIBC ```
INCREASED iron, transferrin, ferritin NORMAL/DECREASED TIBC
79
Gold standard of dx for hereditary hemochromatosis Tx
Liver biopsy: increased liver parenchymal hemosiderin Tx: Phlebotomy
80
Coagulopathy of Advanced Liver Disease: (increase/decrease) PT (increase/decrease) albumin (increase/decrease) PTT Tx:
Increased PT Decreased albumin Increased PTT (in advanced disease) Tx: Fresh Frozen Plasma
81
T/F: Increased association w/ EBV seen in Non-Hodgkins Lymphoma
False. In Hodgkins Disease. Rare in most NHL.
82
Antibodies against RBC's surface causing increased RBC destruction by macrophages, spleen and complement Dx:
Autoimmune Hemolytic Anemia Dx: + Direct Coombs test Cold agglutinin study Peripheral smear: microspherocytes, polychromasia, agglutination of RBCs
83
+ Coombs distinguishes ____
Autoimmune Hemolytic Anemia from Hereditary Spherocytosis
84
Warm agglutinin =___ , causes ___. Etiology: Cold agglutinin =___, causes ___. Etiology: Seen in ___
Warm = IgG Ab --> SPLENIC macrophage RBC destruction via phagocytosis Etiology: SLE*** MC (Autoimmune d/o) Cold = IgM Ab -->INTRAVASCULAR complement-mediated RBC lysis, especially in COLD temperatures Etiology: May follow infection (Mycoplasma**, EBV**) Seen in Autoimmune Hemolytic Anemia
85
Tx of Autoimmune Hemolytic Anemia
Warm: Steroids**, immunosuppressants, splenectomy (removes site of RBC destruction) Cold: avoid cold exposure**, warm fluids. Rituximab
86
Osmotic fragility test used to dx ____ Coombs test +/- Peripheral smear: (Hyper/normo/hypo)chromic (macro/normo/micro)cytosis
Hereditary spherocytosis Coombs test negative Smear: 80% spherocytes, HypERchromic MICROcytosis
87
Hereditary spherocytosis: Autosomal ___ intrinsic hemolytic anemia Causes defect in ____ --> ____ and ___ --> Increased RBC hemolysis in ___.
Autosomal dominant Defect in RBC membrane/cytoskeleton --> cell fragility and sphere-shaped RBCs --> hemolysis in SPLEEN
88
Treatment of choice of Hereditary spherocytosis in severe disease
Splenectomy* Folic acid not curative but helpful
89
Von Willibrand disease will have increased ____.
Bleeding time | PTT

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