Hematology Flashcards
1
Q
What is anemia
A
Reduction own RBC mass or blood Hgb conc. (
2
Q
Lab test for anemia
A
CBC w/ diff
Peripheral blood smear
Reticulocyte count
3
Q
Normocytic normochromic anemia
A
Anemia of chronic disease
4
Q
Microcytic hypochromic anemia
A
Fe deficiency
Thalassemia
Pb toxicity
5
Q
Microcytic anemia
A
B12 def
Folate def
6
Q
Signs of anemia
A
Acute: lethargy, tachycardia, pallor, irritability, poor oral intake
Chronic: Few or no sxs
7
Q
Anemias associated w/ bone marrow failure
A
Fanconi anemia
Acquired plastic anemia
8
Q
anemia due to inherited autosomal recessive bone marrow failure
A
Fanconi anemia
9
Q
Clinical presentation of fanconi anemia
A
Pancytopenia
Abnormal pigmentation skin
Short stature
Skeletal malformation
10
Q
Lab findings of fanconi
A
Thrombocytopenia/ leukopenia
Anemia
Bone marrow hypoplasia-replaced w/ fat cells (aplastic anemia)
11
Q
Tx Fanconi
A
- Supportive tx; Anemia, transplant for thrombocytopenia, neutropenia (may be susceptible for infection)
- Hematopoietic stem cell transplant
12
Q
Acquired aplastic anemia
A
peripheral pancytopenia w/ a hypo cellular bone marrow
50% cases idiopathic
Other causes: meds, toxic exposure, viruses
13
Q
Clinical presentation of acquired aplastic anemia
A
Weakness, fatigue, pallor, frequent infections, purpura, petechiae, bleeding
14
Q
Lab findings for Aplastic anemia
A
- Normocytic anemia
- Low WBC w/ neutropenia
- Thrombocytopenia
- Low reticulocyte count
15
Q
Complications of aplastic anemia
A
- Overwhelming infection
- Severe hemorrhage
16
Q
Aplastic anemia tx
A
- Supportive care
- Referral
- Stop offending agent
- Abx
- RBC transfusion
- Platelet transfusion
- Immunosuppressants
- HSCT
17
Q
Most common nutritional deficiency in children, prevalently of African American and hispanic desent
A
Iron deficiency anemia
18
Q
Signs of IDA
A
Pallor
Fatigue, irritability
Delayed motor debt
Hx of pica
19
Q
When does screening for anemia need to be done
A
12mos of age (Hgb conc & risk factor assessment)
20
Q
Risks for IDA
A
- Low socioeconomic status
- Premature/low birth weight
- Lead exposure
- Exclusive breast feeding beyond 4mos
- Weaning to whole milk/foods that don’t contain iron
- Feeding probs
21
Q
Lab findings for IDA
A
- Microcytic hypo chromic anemia
- Hgb
22
Q
IDA tx
A
- Hgb 10-11mg/dL @ 12mo visit; monitor closely, recheck Hgb in 1mo
- Iron 6mg/kg/d, 3x qd
23
Q
Types of megaloblastic anemia
A
- Vit B12 def
- Folic acid def
24
Q
Causes of Vit B12 def
A
- Intestinal malabsorption
- Dietary insufficiency (req animal protein)
25
Causes of folic acid def
- Increased folate req (rapid growth, chronic hemolytic anemia)
- malabsorptive synd (celiac)
- Inadequate dietary intake
- Meds (methotrexate)
26
Clinical presentation Megaloblastic anemia
Pallor
Glossitis
*Older can w/ Vit B12 = parenthesis, weakness, unsteady gait, decreased vibratory sense/proprioception
27
Lab findings Megaloblastic anemai
Elevate MCH & MCV
Low folic acid +/- vit B12
Hyper segmented neutrophils
Large oval RBCs (macro ovalocytes)
28
Tx megaloblastic anemia
Vit B12/Folate supplements
| Must treat Vit B12 Def or neurological def will be permanent
29
Which diseases are examples of congenital hemolytic anemias
- Hereditary spherocytosis
- Thalassemia
- Sickle cell disease
- G6PD
- Pb poisoning
30
Hereditary spherocytosis
* Red cell membrane defect
* Pt-Jaundice, splenomegaly, gall stones
* Lab-spherocytes on peripheral smear
* increased osmotic fragility
* Dx-Supportive care +/- RBC transfusion
* possible splenectomy
31
Thalassemia
* alpha or beta, more deletions = more severe, microcytic hypochromic
* Lab-Hgb Electrophoresis
* Rx-Fe monitoring/chelation, splenectomy, HSCT (beta)
32
Sickle cell
* Homozygous HbSS, vasoocclusion=pain
* Pt-splenomegaly, asplenia, increased risk of sepsis, delayed puberty
* Hgb Electrophoresis
* Rx-Avoid precipitating factors, supportive, hyrdoxyurea, stem cell transplant
33
Disease characterized by howell jolly bodies
sickle cell anemia
34
G6PD def
* X linked recessive red cell enzyme, causes hemolytic anemia, highest among African, Medit, Asian. episodic hemolysis due to exposure to oxidant stress, drugs and food substances
* Pt-neonatal jaundice, hyperbilirubinemia, pallor
* Bite like deformities and Heinz bodies on peripheral smear
* Tx-supportive, avoidance of certain food/drugs
35
Pb poisoning
* Lead based paint exposure (old home), hemolytic, normocytic
* Labs-basophilic stippling
* Tx-chelation
36
Disease characterized by heinz bodies
G6PD def
37
Disease characterized by Basophilic stippling
Lead poisoning
38
Two types of polycythemia
Primary
| Secondary
39
Primary polycythemia
* Congenital; many cells = risk of thrombosis, only RBCs affected
* High Hgb (27g/dL)
* splenomegaly, plethora (ruby red appearance), lethargy, HA
* Rx: Phlebotomy
40
Secondary polycythemia
* Occurs in response to hypoxemia (cyanotic congenital heart disease, chronic pulmonary disease)
* Rx: Treat underlying condition, phlebotomy
41
What tests do we do when we are concerned about a bleeding disorder
* CBC (platelet count)
* Peripheral smear
* PT/INR, aPTT
* Bleeding time
42
Normal platelet count
150K-400K
43
Platelet count that indicates spontaneous bleeding
44
What does PT (prothrombin time) measure
Extrinsic and common pathway (I, II, V, VII, tissue factor)
45
What does PTT/aPTT (activated partial thromboplastin time) measure
Intrinsic and common pathway (I, II, V, VIII, IX, X, XI, XII)
46
INR measures?
used to monitor warfarin (coumadin)
47
Bleeding time measure?
Platelet dysfunction (time for hemostasis)
48
in what diseases is bleeding time prolonged
VWB, severe thrombocytopenia
49
What is the most common acute bleeding disorder of childhood
Idiopathic thrombocytopenia purpura (ITP)- 2-5yo (post viral infection)
50
Pathophysiology of ITP
immune mediated attack of its own platelets
51
How does ITP present
- Petechiae
- Ecchymosis
- Epistaxis
52
ITP lab findings
- Thrombocytopenia
- Normal WBC
- Normal Hgb
- PT and aPTT normal
53
ITP diagnosis
dx of exclusion
54
ITP tx
- Avoid NSAIDs/Asprin
- Bleeding precautions
- Prednisone
- IVIG
- Splenectomy
- Observe in asymptomatic chn
55
Most common inherited bleeding disorder
Von Willebrand disease
56
Pathophysiology of VWD
impaired action of or decreased level of VWF = impaired platelet adhesion to endothelium. 3 types; Type 1 most common
57
Clinical presentation of VWD
- prolonged bleeding time from mucosa; epistaxis, menorrhagia, GI
- Easy bruising
58
Lab findings of VWD
- Normal PT
- Prolonged/norm aPTT
- Norm decreased factor VIII
- Norm/decreased VWF
- prolonged bleeding time (diff than Hemophilia)
59
Tx VWD
- Desmopressin
| - VWF replacement therapy
60
What is the most common hemophilia
Hemophilia A (factor VIII def)
61
Christmas disease
Hemophilia B (factor IX)
62
Is hemophilia X linked
Yes, M>F
63
Clinical presentation of Hemophilia
- Bleeding
- Mild: bleed in response to trauma/injury, not clinically apparent till later in life
- Severe: sever bleeding, spontaneous bleeding, earlier age of first episode
64
Common site of bleeding in hemophilia
Joints and muscles (hemarthrosis)
65
Lab findings of hemophilia
- Normal platelet count, PT, bleeding time
- Prolonged aPTT
- Normal VWF
66
Tx for hemophilia
- Desmopressin (hemophilia A)
| - Factor replacement (VIII and IX)
67
What diseases are acquired bleeding diseases
- DIC
- Liver disease
- Kidney disease
68
Acquired bleeding syndrome xtized by hemorrhage and microvascular thrombosis
DIC (disseminated intravascular coagulation)
69
What triggers DIC
- sepsis
- trauma/tissue injury
- malignacies
70
Clinical manifestations of DIC ***
- Shock
- Diffuse bleeding tendency
- Evidence of thrombotic lesions
71
Lab findings for DIC
- Decreased platelet count and fibrinogen
- Prolonged aPTT and PT
- Elevated D-dimer and fibrin degradation pdts
72
Tx for DIC
- Identification of triggering event
- Replacement therapy
- Anticoagulation therapy`
73
Acquired bleeding disorders
- Vitamin K deficiency
| - Liver disease
74
Where is prothrombin, fibrinogen and factors V, VII,IX, X, XII and XIII made
Liver
75
Which factors are dependent on vitamin K
II, VII, IX, X (depressed neonatal activity of Vit K dependent factors)
76
Lab findings for acquired bleeding disorders
Normal platelet count in Vit K def and Normal-low in liver disease
Prolonged PT, aPTT
77
Tx for acquired bleeding disorders
- Treat underlying condition
| - vit K at birth
78
Inherited thrombotic disorders
- Protein C def
- Protein S def
- Antithrombin Def
- Factor V leiden mutation
79
Thrombotic disorder in which activated protein C inactivates activated factors V and VII
Protein C deficiency (Tx w/ Heparin) -
80
Danger in protein C def
warfarin induced necrosis
81
What is the role of protein S
Co-factor for protein C that facilitates the action of activated protein C
82
Factor V leiden mutation pathophys
point mutation that makes factor V resistant to inactivation by activated protein C`
83
What does factor V leiden mutation increase the risk of
-VTE (2-7fold), 35 fold in heterozygous taking oral contraceptives, 80 fold in homozygous
84
pathophys of antithrombin def
Inhibitor of thrombin (efficiency of heparin may be diminished)
85
Tx for thrombotic disordera
- Anticoagulation prophylaxis
| - Anti coat agents (unfractionated heparin, low molecular weight heparin, warfarin)
86
How long should you treat an adult post first VTE episode
3mos
87
Inflammation of deposits of IgA complexes in the GI, kidneys, skin (small vessel vasculitis) often presenting with hematuria
Henoch-Schonlein purpura (HSP)
88
What is the most common type of small vessel vasculitis
HSP
89
Clinical presentation of HSP
- Palpable Purpura
- arthritis/arthralgia
- Abdominal disease
- Renal disease
90
Lab findings of HSP
- Normal/elevated platelet count
- Hematuria (UA)
- Hemoccult
- Elevated serum IgA
- Elevated antistreptolysin O
91
Tx HSP
-Supportive (good prognosis)
