Hematology Flashcards
(102 cards)
1
Q
Aplastic Anemia biopsy results
A
Hypocellular BM with increased fat content and morphologically normal Hematopoietic cells
2
Q
Aplastic Anemia further workup
A
- Cytogenetic analysis - r/o myelodysplastic syndr
- PNH screen
- B12 / folate levels
- Hepatitis serology
- HIV testing
3
Q
Aplastic Anemia tx
A
- withdrawal of potentially causative agent
- antithymocytes globulin + cyclosporine (dec transfusion requirement)
- SCT (for young)
4
Q
Common cause of secondary pure red cell aplasia
- malignancy
- infectiuos
A
- Large granular lymphocytes leukemia
2. Parvovirus B 19
5
Q
Large granular lymphocytes leukemia often associated with
A
RA / Felty Syndrome
6
Q
PNH dx (2)
A
Lack of FLAER (fluorescein-labeled proaerolysin)
Lack of CD55 and CD 59 on erythrocytes and leukocytes
7
Q
Medication which decreased transfusion requirement and spontaneous thrombosis in PNH is
A
Eculizumab
8
Q
Large granular lymphocytes leukemia Flow Cytometry finding
A
CD57 positive T cells
9
Q
Leukemia LAD + severe cytopenia \+ terminal deoxynucleotidyl transferase - MPO Philadelphia chrom t(9;22)
A
ALL
10
Q
ALL / AML associated Tumor lysis syndrome can be txed with
A
IVF / Allopurinol
Rasburicase rapidly lowers uric acid levels
11
Q
ALL treatment
A
Induction chemo
Intrathecal chemoprophylaxis
HSCT
If Philadelphia chrome + = adjunctive use of bcr-abl inh
12
Q
Auer rods
Gingival hypertrophy
Leukemia cutis
A
AML
13
Q
AML high risk cytogenetics
A
Del (5q)
3q abnormalities
14
Q
AML Tx
A
Chemo
Allogenieic HSCT
Promyelocytic Leukemia = ATRA
15
Q
AML Cx
A
Hyperleukocytosis Synd
- emergent induction chemo or Leukapheresis
16
Q
With Normocytic Anemia and Elevated Reticulocytes consider 3 things ..
A
Erythrocyte loss (bleeding) Erythrocyte destruction (hemolysis) Approp respite to therapy
17
Q
With Normocytic Anemia and Normal or Decreased Reticulocytes consider
A
- Inflammatory dz
- EPO deficiency
- Nutritional deficiency (b12, folate, iron)
- hypometabolism: hypothyroidism or testos def.
- primary hematopoietic d/o: red cell aplasia and myelodysplasia
18
Q
Serum ferritin > —-ng/ml excludes Fe def
A
100ng/ml
19
Q
Serum ferritin < —-ng/ml is consistent with Fe def
A
10-15ng/ml
20
Q
Methylmalonic acid + homocysteine levels increased in …
A
Vit b12
21
Q
Nml Methylmalonic acid + Inc homocysteine levels seen in
A
Folate
22
Q
What else can inc MCV (other than Megaloblastic anemia)
A
Reticulocytosis seen in hemolysis
23
Q
Can Vit B12 def px with neurologic sxs in the absence of anemia or macrocytosis
A
YES
24
Q
Schistocytes are commonly seen in
A
TTP/HUS
DIC
HELLP
Valve leak
25
Spherocytes are commonly seen in (2)
| What test differentiate them?
Autoimmune hemolytic anemia
HS
Direct Coombs
26
Warm-Ab autoimmune hemolytic anemia test to diagnose
Direct Coombs test for IgG
27
Cold agglutinin autoimmune hemolytic anemia test to diagnose
Directt Coombs test for C3 / IgM
28
Warm-Ab autoimmune hemolytic anemia tx
Glucocorticoids or Rituximab
| if unresponsive Splenecotmy
29
Cold agglutinin autoimmune hemolytic anemia tx
Keep warm
| Rituximab
30
TTP tx
Emergent plasma exchange
31
Clinical signs of patient with Acute chest syndrome
CP
New pulmonary infiltrate
Hypoxemia
Fever
32
Hydroxyurea indications (2)
- 3 or more pain crises
| - Recurrent or severe acute chest synd
33
Indications for exchanged transfusion in SS (3)
- Acute stroke
- Fat embolism
- Acute chest syndrome
34
Indication for prophylactic exchanged transfusion in SS (1)
History of ischemic stroke
35
Contraindications to use hydroxyurea (2)
Kidney failure
| Pregnancy
36
Which disease has ELEVATED HgA2 and fetal Hg
B-Thalasemia trait
| (normal in a-thalassemia trait)
37
Potential complications of XRT (4)
1. early onset CAD
2. radiation pneumonitis
3. hypothyroidism (not hyper)
4. development of solid tumors dependent on the radiation field
38
BRAF mutation is commonly seen in which hematologic malignancy
Hairy cell leukemia
39
JAK 2 mutation is seen in
Myeloprolif disorders:
Polycythemia vera
Essential thrombocythemia
Primary myelofibrosis
40
PT / PTT assesses which factors
PT - 2,7,9
| PTT (intrinsic pathway) - 8,9,11,12
41
With pure red cell aplasia - describe red cell morphology (hypo chromic, megaloblastic, normal) and reticulocyte count (increases, decreases, normal)
Normal red cell morphology
| Dec retic count (BM defect)
42
Platelet clumps on peripheral smear caused by EDTA-dependent agglutinin. Repeat plt count using citrated or heparinized tube will lead to normal counts
Pseudothrombocytopenia
43
In immune thrombocytopenia Abs arise in 3 clinical setting
1. Drug induced
2. Dz associated (HIV, hep C, lymphoprolif malignancy)
3. Idiopathic
44
Tx for ITP
Glucocorticoids if
- plt <30
- plt < 50 with bleeding
45
Glucorticoid-resistant ITP tx or Severe bleeding tx
```
IV Ig
Anti-RhD Ig (transient response)
Splenectomy
Rituximab
Thrombopoiesis-stim agents: Romiplostim, Eltrombopag
```
46
Evaluation for secondary Hemostasis
- mixing study (no correction =factor inh)
- thrombin time (conversion of fibrinogen to fibrin)
- excessive fibrinolysis (LOW fibrinogen, HIGH fibrin degradation products and D-dimer)
47
Hemophilia A and B
- inheritance type
- deficient factor
X - linked recessive
A - Factor 8
B - Factor 9
48
Hemophilia A and B tx
Recombintant Factor VIII and IX
Hemophilia A - desmopressin
49
Acquired Hemophilia
- mixing study results
- prolongation of PT/PTT/both
No correction of PTT with mixing study due to acquired Ab to Factor VIII
50
vWD dz screening tests (2)
- prolonged plt fxn analyzer closure time
| - normal / prolonged PTT
51
vWD dz definitive diagnostic tests
vWF Ag level and activity assay
Factor VIII level
von Willebrand multimer (elevated)
52
vWD dz tx
DDAVP
Factor VIII concentrates
No cryoprecipitate - due to infection risk
53
Blood type that is universal donor
Type O neg
54
Cryoprecipitate provides..
Tx of choice in bleeding pts with..
vWF, Factor 8, 13, Fibronectin, Fibrinogen
- hypofibrinogenemia from liver dz
- thrombolytic therapy
- DIC
55
Lupus AC thrombophilia testing
- prolonged PTT
| - does not correct with mixing study
56
Lupus AC thrombophilia Abs
Anticardiolipin Ab
| anti-B2 glycoprotein 1 Ab
57
Tx indicated for pt with MM to reduce the incidence of skeletal related events
Zoledronic acid
| Pamidronate
58
Palliation medication that decreases leukocytosis and splenenomegaly in CML
Hydroxyurea
59
For Essential Thrombocytosis / Polycythemia indication to use Hydroxyurea + ASA
- plt count >1,000,000
- age >60 yo
- CV disease
- previous thrombosis
60
PV testing : is EPO increased or decreased
Decreased
61
Inc Hg and Inc EPO causes
Secondary Erythrocytosis
1. hypoxemia***
2. diuretics
3. anabolic steroid use
4. kidney or liver carcinoma
5. malignancy
62
Ruxolitinib - mech of action
JAK 1 or 2 inhibitor
63
Anemia + spur cells
Liver dz
64
Can Vit B12 deficiency present without anemia but with neurologic deficits
YES
65
With Multiple Myeloma do you need Skeletal survey OR Bone scan
Skeletal survey - lytic lesions
66
Poor prognostic factors for ALL
> 60 yo
Mature B or T cell
t (9;22) translocation
t (4;11)
67
Is protein C deficiency acquired or inherited or both?
Acquired - DIC, pregnancy, liver dz, nephrotic syndrome, Vit K deficiency
Inherited - autosomal dominant
68
Howell-Jolly bodies on peripheral smear typically occur due to (small black pellets of nuclear remnants in the cells)
Splenectomy
69
Delayed hemolytic transfusion reaction is most commonly due to?
And happens when?
Symptoms?
Rh incompatibility or minor Ag mismatches
Happens ~7Ds after transfusion
Anemia, mild fever, mild unconjugated bilirubin
70
Acute hemolytic transfusion reaction most commonly due to?
ABO incompatibility
71
Febrile nonhemolytic transfusion reaction most commonly due to?
Leukocytes in the transfused blood product
72
Acute intermittent porphyria (3 sxs)
Neuropathies
Psych manifestation
Neurovisceral abdominal pain
73
Acute intermittent porphyria underlying pathogenesis
heme biosynthetic pathway
74
Waldenstrom macroglubulinemia is associated with ..
| IgM or IgA elevations
IgM
75
Waldenstrom macroglubulinemia complication?
Tx?
Hyperviscosity syndrome (IgM levels are markedly elevated)
Requires Plasmaphresis
76
4 Myeloproliferative Neoplasms
CML
Essential thrombocythemia
PV
Primary myelofibrosis
77
Skin necrosis with warfarin is associated with what deficiency
Protein C deficiency
78
First diagnostic test in a pt presenting with PV symptoms/signs.
O2 sat to exclude hypoxia
79
How do you differentiate between vWF deficiency and Factor 8 deficiency?
Ristocetin-induced platelet aggregation (RIPA) is reduced in vWF deficiency
80
Sideroblastic anemia is associated with (6)
| ringed sideroblasts; small, blue-staining, iron-containing granules arranged in a ring around the nucleus of RBC
```
MDS
Idiopathic
Alcoholism
Copper deficiency
Lead toxicity
Meds (linezolid, INH)
```
81
First test when suspecting thrombocytopenia secondary to ITP
Repeat platelet count in a citrate collection tube to exclude potential for clumping and confirmation.
82
Patients with sickle cell disease undergoing low- to moderate-risk surgery should receive erythrocyte transfusion targeting a hemoglobin level of -------- before the procedure to avoid complications.
10 g/dL before the procedure to avoid complications
83
Hemochromatosis screening test
Transferrin saturation
> 45% in F
> 50% in M
84
Pseudo-Pelger-Huet anomaly (hypolobulated neutrophil connected to each other) is seen in
Myelodysplastic syndrome
85
MDS BM findings
dysplastic maturation in 1-3 cell lines with hyper cellular BM
+/- ringed sideroblast (ring is iron deposit)
86
MDS cytogenetic syndrome with favorable outcome? and Why?
5 q del
| it responds to Lenalidomide
87
AE of TK inh (etc imatinib) used for CML t (9:22)
Fluid retention
GI toxicity
Rash
88
6 causes of reactive thrombocytosis
```
Fe def anemia
Malignancy
Collagen vascular dz
Infection
Post-splenectomy state
Other MPD
```
89
vWD disease tx
Desmopressin or DDAVP / Stimate
raises plasma vWF and Factor VIII levels by release from the endothelium
```
vWF concentrate (also contains Factor VIII)
Cryoprecipitate transfusion (vWF and Factor VIII)
```
90
Mild platelet dysfunction syndrome:
| Glycoprotein Ib platelet defect leading to problems with adhesion to the endothelium
Bernard-Soulier Syndrome
tx with platelet transfusion
91
Mild platelet dysfunction syndrome:
| Glycoprotein IIb-IIIa defect leading to problems with cross-link fibrinogen (aggregation)
Glanzmann Thrombasthenia
92
Factor VIII Inhibitor tx
activated PCC (prothrombin complex concentrate)
93
Hemophilia B tx
Factor IX concentrate
| FFP
94
Factor X deficiency is associated with which disease
Amyloidosis
95
Factor deficiency that have prolonged PTT but does not have clinical bleeding
Factor XII (Hageman factor)
96
Most common cause of heparin / enoxaparin resistance is
Antithrombin deficiency
97
M spike in MM is made up of which 2 antibodies ?
M spike in MGUS?
> 10% plasma cells with end organ damage ; IgG > IgA
< 10% plasma cells - IgM spike
98
Waldenstrom Macroglobulinemia AKA lymphoplasmatic lymphoma antibody spike
IgM --> pentamer --> hyper viscosity
99
Burr cells AKA echinocytes
| --little projections
CKD
100
Basophilia / Eosinophilia is associated with which disease
Myeloproliferative diseases
101
Episodes of fever, polyserositis, arthritis, erysipeloid rash around the ankles, and elevated acute phase reactants is c/w
Familial Mediterranean fever
102
Familial Mediterranean fever tx
Colchicine
