PANCE Prep- Hematology Flashcards
1
Q
___ presents in adults with a cell lineage of the immature granulocytic cells seen in the peripheral blood e.g. myeloblasts, promyelocytes.
-Auer rods also are commonly seen in this condition
A
Acute myelogenous leukemia (AML)
2
Q
When a patient receives more than one total blood volume (10 units) without replacement of ___, the patient will continue to bleed without administration of ___
A
clotting factors
fresh frozen plasma
3
Q
A positive direct Coombs’ test may be seen in what condition
A
Autoimmune hemolytic anemia
hereditary spherocytosis has negative coombs
4
Q
Hereditary spherocytosis is detected utilizing ___
A
- the osmotic fragility test
2. negative Cooombs
5
Q
___ is indicated in Coumadin overdosage
A
Fresh frozen plasma
6
Q
___ is indicated in type 2 and 3 patients with von Willebrand disease bleeding
___ is indicated in type 1 von Willebrand disease bleeding
A
vWF concentrate
DDAVP** (vasopressin) used to bleeding
7
Q
__ is indicated for patients with Hemophilia A.
A
Factor VIII or cryoprecipitate
8
Q
Once the diagnosis of heparin induced thrombocytopenia is suspected treatment should include:
A
- immediate discontinuation of all forms of heparin
2. treatment with a direct thrombin inhibitor should begin
9
Q
___ is used for heparin overdosage
A
Protamine sulfate
10
Q
What condition is caused by a disorder in the red blood cell membrane?
A
Hereditary spherocytosis
11
Q
What Rh genotype of the mother and fetus puts the baby at risk for hemolytic disease of the newborn?
A
Mother: Rh negative
Fetus: Rh positive
12
Q
Low molecular weight heparin (LMWH) dosage is based on which of the following?
A
Weight
13
Q
___ is the diagnostic study of choice for G6PD deficiency.
A
G6PD assay
14
Q
___ is indicated in the evaluation of hemolytic anemia
A
Hemoglobin electrophoresis
15
Q
An African American male is placed on hydroxychlorquine (Plaquenil) for travel to Africa. Within six weeks he presents complaining of fatigue. CBC shows anemia. Which of the following is the diagnostic study of choice in this patient?
A
G6PD assay
16
Q
CML (chronic myeloid leukemia) patients typically present with what physical exam finding
A
splenomegaly
17
Q
Hereditary spherocytosis presents with what type of anemia
A
a normocytic, normochromic anemia and many spherocytes.
18
Q
___ presents with a prolonged aPTT and normal platelet count and function.
A
Hemophilia A
19
Q
An 18 year-old woman presents to the clinic complaining of fatigue. She reports a past history of lifelong frequent nosebleeds and bleeding gums. She also has menorrhagia. Her mother and maternal grandfather have a similar bleeding history. Initial lab results are as follows: WBC 9,500/mm3, Hgb 10.9 g/dL, HCT 33%, MCV 69 fL, MCHC 26 pg and platelets 284,000/mm3. Which of the following tests should be ordered to evaluate this patient’s diagnosis?
A
Bleeding time and platelet aggregometry
*presentation is consistent with a congential qualitative platelet disorder, most likely von Willebrand’s Disease, necessitating a bleeding time and evaluation of platelet function.
20
Q
What therapies is recommended for a 13 month-old child with sickle cell disease?
A
Folic acid and penicillin V
*Ferrous sulfate is not globally recommended for patients with sickle cell disease.
21
Q
What Leukemia is most closely associated with the Philadelphia chromosome?
A
chronic myelogenous leukemia
22
Q
___ is used to treat immune-mediated hemolytic anemias
A
Prednisone
Splenectomy- long term definitive tx
23
Q
immune-mediated hemolytic anemias presents with ___ red blood cells.
A
normocytic, normochromic
24
Q
___ is characterized by bleeding from many sites as all coagulation factors are consumed and then broken down, leading to:
- decreased fibrinogen level and platelet count,
- prolonged PT and PTT
- presence of FIBRIN SPLIT products.
A
Disseminated intravascular coagulation
25
____ transports Fe throughout the body. This carrier can be measured indirectly by ____
transferrin
TIBC
26
Describe transferrin, TIBC and ferritin levels in iron deficiency anemia and anemia of chronic disease
Fe deficiency- Increased transferrin, increased TIBC, decreased ferritin
anemia of chronic disease- Decreased transferrin, decreased TIBC, and increased ferritin
*both have decreased serum Fe
27
HgbS on hemoglobin electrophoresis
sickle cell anemia (hemolytic)
28
Microcytic anemia with normal/increased serum Fe or no response to Fe tx
Thalassemia
29
How to differentiate alpha and beta thalassemia
alpha: hemoglobin electrophoresis: normal Hgb ratios of HbgA, A2, and F
beta: hemoglobin electrophoresis: DECREASED HgbA, INCREASED HgbF, increased HgbA2,
30
G6PD is associated with what?
1. sulfa drugs (plaqunil, UTI meds- Bactrim)
2. fava beans
3. infections
31
TTP Pentad
1. thrombocytopenia
2. hemolytic anemia
3. kidney damage
4. Neurologic sx*
5. Fever*
32
HUS Triad
1. thrombocytopenia
2. hemolytic anemia
3. kidney damage* (more so than TTP)
33
- Rouleaux Formation
- RBCs stick together like "stack of coins"
- Increased ESR
- high protein
- Multiple Myeloma
- Infections
34
Bite cells (degmacytes) are seen with:
thalassemia, G6PD
35
Schistocytes (fragmented RBCS) are seen w/
Hemolytic anemias (DIC, TTP, HUS)
36
Keratocytes (helmet shaped RBCs) are seen w/
TTP, HUS, DIC, prosthetic valves
37
Echinocytes "Burr cells" are seen w/
Uremia
38
Hypersegmented neutrophils are seen w/
B12**** and folate deficiencies
39
Presentation:
- *neurological sx, paresthesias (stocking and glove-like paresthesia), gait abnormalities, memory loss, dementia (acute or permanent)
- diarrhea, glossitis, anorexia
- macrocytic anemia
B12 (cobalamin) deficiency
TX: IM B12- watch for signs of HYPOKALEMIA
40
How do you dx B12 and folate deficiency
- Peripheral smear: MCV >115, hypersegmented neutrophils
- increased homocysteine, increased methylmalonic acid*
- + Schilling test
*normal methymalonic acid with folate def.
41
Causes of macrocytic anemia
1. B12 (cobalamin) def.
2. Folate def.
3. ETOH abuse
4. liver disease
5. hypothyroidism
42
Causes of microcytic anemia
1. iron def.
2. alpha/beta thalassemia
3. early anemia of chronic disease (ACD)
4. lead poisoning
5. sideroblastic anemia
43
Presentation:
- pagophagia, pica, angular cheilitis, koilonychia
- microcytic, hypochromic anemia
Iron def. anemia
TX: ferrous sulfate 325mg orally daily
* Vit. C increases Fe absorption
* see increase in reticulocyte count w/in 7 days
44
dysphagia + esophageal webs + atrophic glossitiis + Fe def.
Plummer-Vinson syndrome
45
How do you dx Fe def. anemia
Decreased ferritin, increased TIBC, decreased serum FE
- Increased RDW*,
- decreased transferrin saturation <15%
46
Presentation:
abdominal pain w/ constipation, neurologic symptoms (ataxia, fatigue, learning disabilities, coma, shock, metabolic acidosis
-basophilic stippling and ringed sideroblasts
lead poisoning anemia (plumbism)
47
How do you dx lead poisoning anemia (plumbism)
Peripheral smear: microcytic, hypochromic anemia w/ BASOPHILIC STIPPLING and RINGED SIDEROBLASTS
- Increased serum lead and serum Fe,
- Xray: "lead lines"
48
Normally after ___ old, adult Hgb is predominant Hgb produced
6 months (HgbA- 2 alpha and 2 betas)
49
Think ___ if microcytic anemia w/ normal/increased serum Fe or no response to Fe tx
Thalassemia
50
How do you dx thalassemia
- hypochromic, microcytic anemia w/ normal Fe
- Perpherial smear: TARGET cells, teardrop cells
- HEINZ bodies in HgbH disease
- Hgb Electrophoresis****
51
G6PD affects most commonly in
primarily AA males
52
how do you dx sickle cell disease
decreased Hgb, HCT and increased reticuolocytes
-sickled erythrocytes +/- Howell-Jolly bodies**
Hgb electrophoresis*
53
Tx of sickle cell disease
1. pain control: IV hydration and oxygen in pain crisis
2. Hydroxyurea: reduces freq. of pain crises
3. Folic acid
4. immunize for S. pneumococcus, H. influena and N. meningoccus *SHiN)
54
Common presentations of sickle cell disease
1. Dactylitits MC 1st presentation at 6-9 months
2. Osteomyelitis (esp. Salmonella)
3. Functional asplenia
55
Dark cola-colored urine during the nigh tor early AM w/ partial clearing during the day
-thrombosis ex. hepatic, mesenteric
paroxysmal nocturnal hemoglobinuria
DX: flow cytometry best screening test*
TX: Eculizumab
56
Primary hemostasis involves __
| Secondary hemostasis involves __
Primary hemostasis- platelets form a plug at site of vascular injury: platelet adhesion activation and aggregation
Secondary hemostasis- clotting factors respond in a cascade to form fibrin strands which strengthens the platelet plug
57
What does PT and PTT measure and give examples of things that prolong them
PT: EXTRINSIC factor pathway: (7 and 10**)
ex. heparin, DIC< vWD, hemophilia A and B
PTT: INTRINSIC: (8, 9, 11, 12**)
ex. warfarin, Vit K def, DIC
58
heparin OD antidote:
| warfarin OD antidote:
heparin: protamine sulfate
warfarin: vit K
59
Decreased AdamTS12
primary TTP
tx: plasmapheresis
60
Seen predominately in children (usually preceded by E. coli O157:H7, shigella or salmonella gastroenteritis:
-thrombocytopenia, microangiopathic hemolytic anemia, kidney failure (uremia)
HUS (hemolytic uremic syndrome)
TX: observation / IV fluids to maintain renal perfusion
- plasmapheresis if severe
* *Antibiotics worsen the condition
61
Etiologies of DIC
1. infections (gram neg. sepsis MC)
2. malignanices
3. Obstetrics
4. massive tissue injury
62
how do you DX DIC
1. Increased thrombin, decreased fibrinogen, increased PTT/PT/INR,
2. increased d-dimer (increased fibrinolysis)
63
TX: DIC
tx underlying cause
| 2. FFP if severe bleeding
64
- BIMODAL peaks at 20 and again at 50y/o
- Associated with EBV- MC in males
- PAINLESS lymphadenopathy but may develop painful lymph nodes w/ ALCOHOL
Hodgkin Lymphoma
DX: biposy: REED-STERNBERG cell pathognomoic****
mediastinal lymphadenopathy
TX: radiation +/- chemo
**highly curable compared to NHL
65
- PAINLESS lymphadenopathy peripheral MC- extranodal sites common: GI, skin, CNS MC
- abdominal pain
Non-hodgkin lymphoma
tx: unpredictable course
- follicular: Rituximab: ab vs CD20 on B cells
- Diffuse large B cell: chemo
66
Describe presentation of multiple myeloma
"Bones BREAK" or CRAB
-(Ca elevated, Renal failure, Anemia, Bone osteolytic lesions)
Bone pain: osteolytic, destructive lesion
Recurrent infections from leukopenia
Elevated Calcium (hypercalcemia)
Anemia
Kidney failure (Bence Jones proteins in urine)
67
DX and tx of multiple myeloma
DX:
- serum protein electrophoresis: monoclonal (M) protein spike
- urine protein electrophoresis: Bence-Jones proteins*
- CBC: ROULEAUX formation and increased ESR
- Skull radiograph: punched-out lytic lesions
* **BM biopsy: plasmacytosis <10%
TX: autologous stem cell transplant is definitive tx +/- chemo
68
MC childhood malignancy and common with Downs syndrome
Fever MC sx
CNS sx, hepatosplenomegaly, lymphadenopathy
ALL
DX: BM: hypercellular w/ >20% blasts
TX: combo chemo
69
MC leukemia in adults
- fatigue, painful lymphadenopathy
- SMUDGE cells on peripheral smear
CLL (B cell)
TX: observe if indolent, Fludarabine chemo if progressive
70
lab abnormalities and tx of tumor lysis syndrome
hyperuricemia, hyperkalemia, hypocalcemia, hyper phosphatemia, acute renal failure
allopurinol, IV fluid
71
What leukemia is associated with Philadelphia chromosome
CML (Philadelphia CreaM cheese)
72
Overproduction of all 3 myeloid cell lines- lymphocyte line normal
- JAK2 mutation
- hypervisocity or thrombosis
- Splenomegaly,
Polycythemia vera (primary erythrocytosis)
73
increased iron, ferritin, and transferrin
- liver dysfunction
- HF, cardiomyopathy, arrhythmias
- hypogonadism
- pancreatic insufficiency
- Metallic or Bronze skin**-- bronze diabetes**
```
Hereditary hemochromatosis
(excessive iron deposition in heart, liver, pancreas and endocrine organs)
```
DX: liver biopsy is gold standard-- hemosiderin
tx: phlebotomy
74
MC inherited caused of hypercoagulability
Factor V leiden
75
MC clotting disorders
Factor V leiden
Protein C def.
Antithrombin III Def.
76
Pernicious anemia is due to deficiency in what
intrinsic factor (secreted by parietal cells)
77
MC cause of hypercalcemia in malignancy
Increased production of parathyroid hormone-related peptide (PTHrP)
*MC CA- lung, breast CA and myeloma
2nd MC cause is osteolytic metastasis (NOT OSTEOBLASTIC)
78
PMH: miscariages
| presents w/ clot, lacy erthematous rash on extremities
Antiphosopholipid antibody syndrome
DX: anticardiolipid antibodies, lupus anticoagulant, and anti-beta 2 glycoproteins
79
Presentation:
- 70y/o pt.
- CBC: leukocytosis and lymphocytosis (usually found incidentally on ROUTINE bloodwork)
- 1/3 develop autoimmune hemolytic anemia, and 5% develop ITP
Chronic lymphocytic leukemia (B Cell)
*presents with a WBC count greater than 20,000/microliter and absolute lymphocyte count of greater than 5000/microliter
80
Spontaneous hemarthroses are pathognomonic for:
Hemophilia A and B
81
What is the most serious delayed toxicity that is seen w/ the anthracycline drug class?
cardiomyopathy
82
Bleomycin used in Hodgkin's lymphoma and testicular CA has the known toxicity of ___
Pulmonary toxicity
83
Common findings and tx of Primary polycythemia
1. Elevated Hgb and HCT
2. Elevated platelet- thrombosis/hypervisocity
3. Splenomegaly
4. facial plethora (flushed face*)
5. JAK2 mutation
6. engorged retinal veins
TX: phlebotomy
(diuretic use is suggestive of spurious PV)
84
4 main common types of myeloproliferative neoplasms
1. polycythemia vera (PV)
2. essential thromboytosis
3. CML
4. primary idiopathic myelofibrosis
85
Presentation:
- Bleeding gums, epistaxis
- NO splenomegaly
- Hx of URI
- Purpura or petechiae
- Low platelets
Immune thrombocytopenia purpura (ITP)
TX: steroids***** +/- IVIG
*transfusion or splenectomy if platelet count drops below 5,000-10,000
TX in Adults: steroids or Rituxan and Eltrombopag are approved for ADULTS ONLY
86
If left untreated, Vit. B12 def. can cause
Irreversible neuropathy
87
Vit. B12 is produced by ___ and absorbed in ___
parietal cells
terminal ileum
88
- Decreased WBC, RBCS and platelets (pancytopenia)
- BM biopsy: hypoplastic marrow, NO blasts
- Causes: radiation exposure, chemical exposure to pesticides or solvents, viruses, paroxysmal nocturnal hemoglobinuria and sulfa compounds
aplastic anemia
*if farmer patient think aplastic anemia
89
How do you dx Von Willebrand's Disease
1. S/S: Mucocutaneous bleeding: easy bruising, epistaxis, gums GI, menorrhagia
2. decreased vWF levels
3. +/- prolonged PTT
* **** PTT prolongation worsen w/ aspirin**
4. Decreased ristocetin activity test gold standard**
MC bleeding disorder- inherited autosomal dominant
TX: Vasopressin or DDAVP
90
Presentation:
| platelet count starts to drop after being on coumadin and heparin and start developing thromboses
Heparin-induced thrombocytopenia (HIT)
*caused by autoantibodies of platelet factor 4 (PF4)
91
Myelodysplastic Syndrome can progress to ___ in select patients
acute leukemia
92
Packed red cell transfusions (PRBCs) can cause temporary ___
Risk of iron overload has been recognized after packed red blood cell transfusion of __ units
HYPERkalemia
more than 20 units
93
__ products carry the highest risk of tranfusion-related acute lung injury (TRALI) among all blood products
plasma, apheresis platelets, and whole blood
TX: oxygen and possibly ventilation
*occurs w/in 6 hours of transfusion (presentation: hypoxia, pulmonary infiltrates, hypotension, cyanosis)
94
One unit of Packed red cell transfusions (PRBCs) is expected to raise the hemoglobin ___
1 Hgb
95
The following peripheral smears match what diseases?
1. Target Cells
2. Howell-Jolly bodies
3. Heinz Bodies
4. Rouleaux formation
5. Poikilocytosis
6. Bence Jones protein in urine
1. Target Cells: liver dz, hemoglobinopathy like thalassemia
2. Howell-Jolly bodies: post-splenectomy, splenic dysfunction, sickle cell
3. Heinz Bodies: G6PD
4. Rouleaux formation (stacked coin appearance of RBCS): myeloma
5. Poikilocytosis: extreme iron deficiency
6. Multiple myeloma
96
Presentation:
- Hx of RA
- Splenomegaly and neutropenia
Felty's syndrome
DX: Clinical, CBC, +/- BM biopsy to r/o other causes
97
- Prolonged PTT
| - Mixing study of blood to normal serum results in persistently prolonged PTT
- lupus anticoagulant
- Factor 8 inhibitors
- Contamination w/ heparin
* PTT stays prolonged= presence of an inhibitor
* if PTT corrects w/ mixing study= deficiency of one of the factors (12, 11, 9, 8)
98
What med is the best choice in patients on chemotherapy to help minimize the risk of neutropenia
Pegfilgrastim (long acting colonystimulating factory (CSF) support)
99
Sickle cell disease (HgbS) is what type of inherited disease?
autosomal dominant
*genetic counseling and testing should be performed if fhx even if healthy could have sickle cell trait
100
Most common etiologies of Iron deficiency anemia
1. CHRONIC blood loss (not acute)
| 2. Pregnancy- bc of increased production of erythrocytes
101
When using tamoxifen in the treatment of malignancy, one must keep what toxicity in mind?
secondary malignancy- increased risk of uterine cancer
102
What tumor marker can be used in the screening of patients for cancer?
alpha-fetoprotein- used to screen for hepatocellular carcinoma
(screen in those w/ chronic hep B, C and cirrhosis)
*PSA, CEA, CA 19-9 are not sensitive or recommended
103
What is Christmas disease
```
Hemophilia B (factor 4)
-X-linked recessive disease affecting primarily males
```
104
Vitamin K deficiency occurs primarily with ___
primary biliary cirrhosis
105
what is the most common cause of hypercalcemia in malignancy?
osteoblastic lesions from bone metastasis
106
does Hodgkin's lymphoma or non-Hodgkin's lymphoma have Reed-Sternberg cells
Hodgkin's
107
Hodgkin's lymphoma has what type of age distribution?
| There is also an increased incidence of it after exposure to ___
bimodal
EBV
108
Evaluation of ___ can suggest myelodysplastic syndrome (MDS) but ___ is diagnostic
peripheral smear
BM biopsy
109
Transfusion related acute lung injury (TRALI) is rare but potentially fatal reaction to blood product infusions that occurs within __ hours of transfusion. Even though TRALI can happen w/ any blood product, _____ have the greatest risk.
6 hours
plasma blood products, apheresis platelet concentrates and whole blood (rarely used today)
110
Risk of iron overload has been recognized after packed red blood cell transfusions of more than __ units
20
111
packed red blood cell transfusions are known to cause ___ electrolyte disturbance
hyperkalemia (from leakage of K+ out of the RBCs)
112
Name the peripheral smear match with the disease:
1. Target cell
2. Howell-Jolly bodies:
3. Heinz Bodies:
4. Rouleaux formation:
5. Poikilocytosis:
1. Target cell: liver dz, hemoglobinopathy (thalassemia,)
2. Howell-Jolly bodies: s/p splenectomy or asplenic pt
3. Heinz Bodies: G6PD deficiency
4. Rouleaux formation: elevated plasma proteins ie. myeloma
5. Poikilocytosis: Extreme iron deficiency
113
PTT is sensitive to what factors?
XII, XI, IX, VIII
114
When mixing a pts prolonged PTT blood w/ 1:1 ratio w/ normal donors blood what does it mean if:
1. PTT corrects:
2. stays prolonged:
1. corrects= def. of one of the factors (8, 9, 11, 12)
| 2. prolonged= a presence of an inhibitor (ie. lupus anticoagulant, factor VIII inhibitors, contamination w/ heparin)
