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Hematology Flashcards

1
Q

Definition of anemia

A

Reduction in RBC mass or blood Hb conc 2 standard deviations below normal for age and sex
<11 for all kids 6 mos-5 yrs

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2
Q

Reasons for normocytic, normochromic anemia

A

Anemia of chronic disease

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3
Q

Reasons for microcytic, hypochromic anemia

A

Iron deficiency, thalassemia, lead intoxication

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4
Q

Reasons for macrocytic anemia

A

Vitamin B12 and folate deficiency

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5
Q

Acute signs and sxs of anemia

A

Lethargy, tachycardia and pallor

Infants might be irritable and have poor oral intake

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6
Q

Types of bone marrow failure

A

Fanconi anemia and acquired aplastic anemia

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7
Q

What is Fanconi anemia?

A

Inherited bone marrow failure syndrome (autosomal recessive) due to defective DNA repair
Usually first 5 yrs of life

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8
Q

Manifestation of Fanconi anemia

A

Progressive pancytopenia
May have abnormal pigmentation of the skin, short stature, skeletal malformations
Increased malignancies

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9
Q

Lab findings of fanconi anemia

A

Thrombocytopenia or leukopenia (usually seen before the anemia)
Severe aplastic anemia
Bone marrow hypoplasia or aplasia

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10
Q

What is fanconis often misdiagnosed as?

A

ITP

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11
Q

Treatment for fanconis

A

Supportive

HSCT

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12
Q

Prognosis of fanconis anemia

A

Pts at high risk of developing myelodysplastic syndrome or AML
Many die of bleeding, infection or malignancy in adolescence

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13
Q

What is acquired aplastic anemia?

A

Peripheral pancytopenia with a hypocellular bone marrow (looks similar to fanconis)
Mostly idiopathic

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14
Q

Sxs of acquired aplastic anemia

A

Weakness, fatigue, pallor (anemia)
Frequent infections (leukopenia)
Purpura, petechiae and bleeding (thrombocytopenia)

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15
Q

Lab findings in acquired aplastic anemia

A

Anemia usually normocytic
Low WBC with neutropenia
Thrombocytopenia
Low retic count because of bone marrow failure

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16
Q

Leading causes of death in acquired aplastic anemia

A

Overwhelming infection and severe hemorrhage

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17
Q

Tx of acquired aplastic anemia

A

Supportive care, referral
Abx for infection
Transfusions
HSCT blah blah

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18
Q

What is the most common nutritional deficiency in kids?

A

Iron deficiency anemia

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19
Q

When is iron deficiency anemia more prevalent?

A

African Americans and Hispanic children

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20
Q

When is screening for anemia performed?

A

At 12 mos (determine hb conc and assess risk factors)

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21
Q

Risks of iron deficiency anemia

A

Low status
Premature/low birth weight
Lead exposure
Exclusive breast feeding beyong 4 mos without iron supplements
Moving to milk or food without iron
Feeding problems, poor growth, inadequate nutrition

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22
Q

Lab findings in iron deficiency anemia

A

Microcytic, hypochromic anemia
Hb < 11
Ferritin < 12
Usually RDW high

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23
Q

Tx for iron deficiency anemia

A

Hb of 10-11 (close follow)

IDA- iron 6 mg/kg/d with 3 divided daily doses

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24
Q

What causes B12 deficiency?

A 
Intestinal malabsorption (Crohns, ulcerative colitis, Celiac)
Dietary insufficiency (vegans)
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25
What causes folate deficiency?
Increased folate requirements (rapid growth, chronic hemolytic anemia) Malabsorptive syndromes Inadequate dietary intake (rare) Meds
26
Signs of macrocytic anemia
Pallor Glossitis B12- neurologic sxs in older children
27
Lab findings of macrocytic anemia
Elevated MCH and MCV Neutrophils large and have hypersegmented nuclei Macro-ovalocytes Elevated methylmalonic acid in B12 Elevated homocysteine with B12 and folate
28
Tx of macrocytic anemia
Supplementation Must treat the B12 deficiency to treat neurologic probs *tx with folate will fix anemia picture in B12
29
Types of congenital hemolytic anemias
Hereditary spherocytosis Thalassemia Sickle cell G6PD
30
Hereditary spherocytosis
``` Red cell membrane defect Hemolytic anemia (jaundice, splenomegaly, gallstones) Spherocytes Increased osmotic fragility Maybe splenectomy ```
31
Thalassemia
Alpha or beta (severity based on gene deletions) Hb electrophoresis for diagnosis Support with RBC transfusion (iron monitoring + chelation, splenectomy, HSCT)
32
Most common sign of sickle cell disease
Vaso-occlusion (pain)
33
Tx of sickle cell disease
Hydroxyurea Tx for painful vaso-occlusive episodes Stem cell transplant
34
What is G6PD deficiency?
Red cell enzyme defect that causes hemolytic anemia | Highest among African, Mediterranean and Asian ancestry
35
Manifestations of G6PD deficiency
Neonatal jaundice, hyperbilirubinemia | Episodic hemolysis due to oxidant stress of infection or certain drugs/foods- pallor, jaundice, hemoglobinuria
36
Peripheral smear on G6PD deficiency
Bite like deformities and Heinz bodies
37
When might you suspect lead poisoning?
Older homes with lead paint
38
Manifestations of lead poisoning
Mild, hemolytic and normocytic anemia | Basophilic stippling on peripheral smear
39
Tx for lead poisoning
Chelation
40
Types of polycythemia
Primary and secondary
41
Familial polycythemia (congenital erythrocytosis)
Only RBCs affected Hb may be as high as 27 Plethora (ruddy complexion) and splenomegaly maybe HA and lethargy
42
Tx of familial polycythemia
Phlebotomy
43
Secondary polycythemia
In response to hypoxemia (cyanotic congenital heart disease like ToF or TGA or chronic pulm disease) Tx: correction of disorder and maybe phlebotomy
44
Initial screening tests for bleeding disorder
CBC Peripheral smear Pt/INR, aPTT Maybe bleeding time
45
Platelet count testing for bleeding disorders
Normal is 150,000-400,000 | Risk of bleeding less than 20,000
46
PT test
Measures extrinsic and common pathways (I, II, V, VII, Xa and tissue factor)- 7 and TF specific
47
PTT/aPTT
Measures intrinsic and common pathways (I, II, V, VIII, IX, X, XI, XII)- 8, 9, 11, 12 specific
48
INR testing
More accurate reflection of PT | Used to monitor Warfarin tx
49
Bleeding time test
Screen test for platelet dysfunction | Prolonged in platelet disorders (von Willebrand) and severe thrombocytopenia
50
What is the most common bleeding disorder of childhood?
Idiopathic thrombocytopenia purpura
51
When does ITP occur?
Often after infection with viruses (immune-mediated)
52
Why does ITP occur?
Immune mediated attack against its own platelets
53
Manifestations of ITP
Multiple petechiae Ecchymosis Epistaxis
54
Lab findings of ITP
Thrombocytopenia Normal WBC Normal Hb PT and aPTT normal
55
How do you diagnose ITP?
Diagnosis of exclusion
56
Tx of ITP
``` Observe when asymptomatic Avoid meds that compromise platelets (ASA, NSAIDs) Prednisone IVIG Splenectomy ```
57
Inherited bleeding disorders
von Willebrand disease | Hemophilia
58
What is the most common inherited bleeding disorder?
von Willebrand disease
59
Genetic problem of von Willebrand disease
Decrease in level or impairment in action of vWF
60
What is vWF?
A protein that binds to factor VIII and is a cofactor for platelet adhesion to endothelium
61
Presentation of von willebrand disease
Prolonged bleeding from mucosal surfaces (epistaxis, menorrhagia, GI) Easy bruising
62
Lab findings of von Willebrand disease
``` Normal PT Prolonged or normal aPTT Normal or decreased factor VIII Normal or decreased vWF Prolonged bleeding time (differentiate from hemophilia) ```
63
Treatment of von willebrand disease
Desmopression (causes release of vwf and factor VIII from endothelial stores) vWF replacement therapy
64
Types of hemophilia
A- factor 8 deficiency (most common) B- factor 9 deficiency (christmas disease) male most common
65
Presentation of hemophilia
``` Bleeding from impaired hemostasis Common sites into joints and muscles Spontaneous hemarthrosis (into joint)- severe disease ```
66
Presentation of mild hemophilia
Bleed in response to injury/trauma or surgery | May not be clinically apparent until later in life
67
Presentation of severe hemophilia
Severe, spontaneous bleeding | Earlier age of first episode
68
Lab findings of hemophilia
Normal platelet count, PT and bleeding time Prolonged aPTT Normal vWF
69
Tx of hemophilia
Desmopression: hemophilia A | Factor replacement: both (to achieve sufficient hemostasis)
70
What is DIC?
Hemorrhage and microvascular thrombosis (triggered by sepsis, trauma/tissue injury, malignancies)
71
Signs and sxs of DIC
End-organ dysfunction (shock) Persistent oozing from needle punctures or other invasive procedures (common) Hematuria, melena, purpura, petechiae Purpura fulminans, major vessel thrombosis
72
Lab findings of DIC
*elevated D dimer and fibrin degradation products Decreased platelets Prolonged aPTT and PT Decreased fibrinogen
73
Tx of DIC
ID and treat triggering event Replacement therapy for consumptive coagulopathy Anticoagulant therapy
74
What is synthesized in the liver?
Prothrombin, fibrinogen | Factors V, VII, IX, X, XII and XIII
75
Which are the vitamin K dependent factors?
II, VII, IX and X
76
Lab findings in liver disease
Platelet count normal in vitamin K deficiency and maybe low in liver disease Prolonged PT, aPTT
77
Tx of liver disease
Treat underlying condition | Vitamin K at birth
78
Inherited thrombotic disorders
Protein C/S deficiency Antithrombin deficiency Factor V leiden mutation
79
What is Protein C deficiency?
Protein C inactivated factors V and VIII | May develop warfarin-induced skin necrosis
80
What is protein S deficiency?
Protein S is a cofactor for protein C (facilitates action of activated protein C)
81
What is factor V leiden mutation?
Point mutation | Factor V polymorphism is resistant to inactivation by activated protein C
82
VTE and factor V leiden mutation
Risk of VTE is increased Among heterozygotes taking oral contraceptives Among homozygotes much higher
83
Antithrombin deficiency
It is a major physiologic inhibitor of thrombin
84
Presentation of antithrombin deficiency
VTE
85
Tx for bleeding disorders
Anticoagulant prophylaxis UFH, LMWH, warfarin First VTE for at least 3 mos (may need long term)
86
What is Henoch-Schonlein Purpura?
Most common type of small vessel vasculitis Mostly boys (2-7) In spring and fall URI often precedes diagnosis (like ITP)
87
Pathophysiology of HSP
Deposition of IgA immune complexes in small vessels of skin, GI tract and kidneys
88
Presentation of HSP
Palpable purpura Arthritis/arthralgias Abdominal pain Renal disease
89
Lab findings of HSP
Usually normal platelet count Antistreptolysin O titer often elevated Hemoccult may be positive Hematuria (sometimes proteinuria)
90
Tx of HSP
Supportive, good prognosis

X Pediatrics 2 (10 decks)

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