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Peds II Unit I > Peds Hematology > Flashcards

Peds Hematology Flashcards

1
Q

What is the actual definition of anemia?

A

A reduction in RBC mass or blood hemoglobin concentration 2 standard deviations below normal for age and sex

Hgb concentration < 11 g/dL for both male and female children aged 6 months to < 5 years

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2
Q

What causes Normocytic, normochromic anemia?

A

Anemia of chronic disease

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3
Q

What causes microcytic, hypochromic anemia?

A

Iron deficiency, thalassemia, lead intoxication

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4
Q

What causes macrocytic anemia?

A

Vitamin B12 and folate deficiency

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5
Q

SSx of anemia

A

Acute:
• Lethargy, tachycardia, pallor
• Infants - irritability and poor oral intake

Chronic:
• May present with few or no symptoms at all

Other findings:
• Jaundice
• Gallstone disease
• Petechiae
•Purpura
• Ecchymosis
•Bleeding
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6
Q

Inherited bone marrow failure syndrome

A

Fanconi Anemia

Autosomal recessive —> defective DNA repair

Majority (75-90%) develop bone marrow failure in the first 10 years of life (usually soon - 1st 5 years)

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7
Q

Clinical manifestations of Fanconi Anemia

A

Progressive pancytopenia

Several congenital malformations
• Abnormal pigmentation of skin
• Short stature
• Skeletal malformations

Increased incidence of malignancies

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8
Q

Condition often initially misdiagnosed as ITP

A

Fanconi Anemia

Misdiagnosed because it begins with Thrombocytopenia or leukopenia before anemia begins

Eventually the anemia —> severe aplastic anemia (low or no production of new cells)

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9
Q

Treatment for Fanconi Anemia

A

Supportive treatment
• Anemia (maybe a transfusion?)
• Thrombocytopenia (platelet transfusion?)
• Neutropenia (meds to stimulate WBC production)

Once all cell lines are affected, requires Hematopoietic Stem Cell Transplant (HSCT)

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10
Q

What is the prognosis for patients with Fanconi Anemia

A

Many succumb to bleeding, infection, or malignancy in adolescence or early adulthood

Patients are at high risk of developing Myelodysplastic Syndrome (MDS) or Acute Myeloid Leukemia (AML)

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11
Q

Peripheral pancytopenia with a hypocellular bone marrow

A

Acquired Aplastic Anemia

~50% of the cases in childhood are idiopathic

Other causes: medications, toxic exposure (insecticides), and viruses (Mono, Hepatitis, HIV)

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12
Q

SSx of Acquired Aplastic Anemia

A

Weakness, fatigue, pallor (duh, it’s anemia)

Frequent or severe infections (duh, leukocytopenia)

Purpura, petechiae, and bleeding (duh, thrombocytopenia)

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13
Q

Lab findings with acquired aplastic anemia

A

Anemia, usually normocytic b/c it’s chronic

Low WBC with marked neutropenia

Thrombocytopenia

Low reticulocyte count b/c bone marrow is failing so few new RBCs are being made

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14
Q

Complications of acquired aplastic anemia

A

Overwhelming infection and severe hemorrhage are leading causes of death

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15
Q

Treatment for acquired aplastic anemia

A

Supportive care

Referral to heme

Stop offending agent if there is one

Abx for infection

RBC transfusions for severe anemia

Platelet transfusions (sparingly)

Immunosuppressant agents

Hematopoietic Stem Cell Transplant (HSCT)

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16
Q

The most common nutritional deficiency in children

A

Iron deficiency

Prevalence is greatest among toddlers aged 1-2 and females of childbearing age

Prevalence higher in African-American & Hispanic children and those living in poverty (low socio-economic status)

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17
Q

SSx of iron deficiency

A 
Varies with severity
Asymptomatic
Pallor 
Fatigue, irritability
Delayed motor development
Hx of Pica
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18
Q

When should screening for iron deficiency be conducted?

A

At 12 months, per AAP guidelines

Determine hemoglobin concentration

Assessment of risk factors

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19
Q

Risk factors for iron deficiency anemia

A

Low socioeconomic status
Prematurity/low-birth weight
Lead exposure
Exclusive breast-feeding beyond 4 months of age w/o iron supplementation
Weaning to whole milk or foods that do not contain iron
Feeding problems, poor growth, inadequate nutrition

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20
Q

Lab findings for iron deficiency

A

Microcytic, hypochromic anemia
Hemoglobin <11 g/dL w/ MCV < 80 and increased RDW
Ferritin < 12 mcg/L (iron stores)

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21
Q

AAP guidelines for the treatment of iron deficiency anemia

A

Hemoglobin of 10-11 mg/dL at 12-month screening visit
• Closely monitor or empirically treat
• Recheck hemoglobin in one month

ID/IDA
• Iron 6 mg/kg/d, 3 divided daily doses

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22
Q

What are the main causes of Vitamin B12 deficiency

A

Intestinal malabsorption (Crohn, Celiac, IBD)

Dietary insufficiency - esp vegans, as B12 is only in animal products

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23
Q

Causes of folic acid deficiency

A

Increased folate requirements (rapid growth, chronic hemolytic anemia)

Malabsorption syndromes

Inadequate dietary intake (rare)

Medications

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24
Q

SSx of megaloblastic anemia

A

Pallor

GLOSSITIS

Older children with B12 can have paresthesias, weakness, or unsteady gait, decreased vibratory sensation and proprioception on neuro exam

***Neurologic SSx ONLY occur with B-12 and not folate deficiency

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25
Q

Lab findings in megaloblastic anemia

A

Elevated MCV and MCH

Low levels of either folic acid and/or vitamin B12

Neutrophils are large and have hypersegmented nuclei

Macro-ovalocytes (large, oval RBCs)

***Elevated methylmalonic acid seen with B-12 deficiency ONLY

***Elevated homocysteine seen with B-12 AND folate deficiency

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26
Q

Treatment for megaloblastic anemia

A

Vitamin B12 and/or folic acid supplementation

Treatment with folate will fix the anemia picture in B-12 deficiency BUT you MUST treat vitamin B-12 deficiency to avoid permanent neurological deficits

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27
Q

Red cell membrane defect leading to hemolytic anemia —> jaundice, splenomegaly, gallstones

A

Hereditary Spherocytosis (HS)

Increased osmotic fragility —> spherocytes

Tx: supportive care +/- RBC transfusion, may need a splenectomy (make sure they have pneumococcal vaccine first

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28
Q

Severity of Thalassemia is related to…

A

The number of gene deletions

Can affect either Alpha or Beta chains of hemoglobin

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29
Q

Microcytic hypochromic anemia of variable severity, due to genetic deletions of alpha or beta chain genes

A

Thalassemia

Dx via hemoglobin electrophoresis

Supportive +/- RBC transfusion
• Iron monitoring and chelation
• Splenectomy may help
• HSCT if severe beta-thalassemia

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30
Q

HbS electrophoresis is used to diagnose…

A

Sickle Cell Disease

Homozygous HbSS —> disease

Vaso-occlusion —> pain (most common Sx)

Chronic hemolysis

Splenomegaly

Splenic infarcts —> functional asplenia

Increased risk of bacterial sepsis

May see growth failure and delayed puberty

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31
Q

Treatment options for Sickle Cell disease

A 
Avoiding precipitating factors
Supportive care
Hydroxyurea
Treatment for painful vaso-occlusive episodes
HSCT
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32
Q

X-linked recessive red cell enzyme defect that causes hemolytic anemia

A

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

Highest incidence among African, Mediterranean, and Asian ancestry

Episodic hemolysis at times of exposure to:
• Oxidant stress of infection
•Certain drugs and food substances (lots of interactions)

33
Q

Clinical manifestations of G6PD

A

Neonatal jaundice, hyperbilirubinemia

Episodic hemolysis (pallor, jaundice, hemoglobinuria)

34
Q

Laboratory findings for G6PD

A

Bite-like deformities and Heinz bodies (denatured hemoglobin) on peripheral smear

35
Q

Treatment for G6PD

A

Supportive treatment

Avoidance of certain foods and drugs known to trigger hemolysis

36
Q

Basophilia stippling (hehehe)

A

Lead poisoning

Common in residents of older homes with lead paint

Anemia is usually mild, hemolytic, and normocytic

Tx: chelation (binds to Pb and allows it to be excreted)

37
Q

Primary polycythemia is also referred to as…

A

Congenital Erythrocytosis Or Familial Polycythemia

Only RBCs are affected

Hemoglobin may be as high as 27 g/dL

Plethora and splenomegaly may be present

Symptoms of headache and lethargy

Treatment = phlebotomy

38
Q

Secondary polycythemia occurs in response to …

A

Hypoxemia - either cyanotic congenital heart disease or chronic pulmonary disease (ie CF, COPD, or asthma)

Treatment: Correction of the underlying disorder, phlebotomy when indicated

39
Q

Initial screening tests for bleeding disorders

A

CBC (specifically, platelets)
Peripheral smear
PT/INR, aPTT
+/- bleeding time

40
Q

What is a normal platelet count?

A

150,000-400,000/mm3 (varies little with age)

Risk of spontaneous bleeding if <20,000

41
Q

What does the prothrombin time (PT) test measure?

A

Measures EXTRINSIC and common pathways

I, II, V, VII, X, and Tissue Factor

42
Q

What does the activated partial thromboplastin time (PTT/aPTT) test measure?

A

Measures INTRINSIC and common pathways (think, it puts another T IN it)

I, II, V, VIII, IX, X, XI, XII

43
Q

INR is a more accurate reflection of …

A

PT

Used to monitor Warfarin treatment

44
Q

Bleeding time test is a measurement of …

A

Time for hemostasis.

Screening test for platelet dysfunction (CBC is just the NUMBER of platelets, doesn’t tell us about how well they work)

Prolonged in platelet disorders (von Willebrand Disease) and severe thrombocytopenia

45
Q

Most common bleeding disorder of childhood

A

Acute ITP (Idiopathic Thrombocytopenic Purpura)

Most frequently in children 2-5 years old

Often follows infection with viruses

Immune-mediated

90% of children with ITP will have a spontaneous remission

Chronic ITP occurs in 10-20% of patients

46
Q

What is the pathophysiology of ITP?

A

Immune mediated attach against one’s own platelets

Autoantibodies bind to platelets —> Platelets are phagocytized primarily by splenic macrophages —> Decreased platelet life span

47
Q

Clinical manifestations of ITP

A

Multiple petechiae
Ecchymosis
Epistaxis

Labs:
Thrombocytopenia
Normal WBC
Normal Hb (unless hemorrhage)
PT and aPTT are normal 
(ALL tests are normal except platelets)
48
Q

Dx of ITP is …

A

A diagnosis of exclusion - rule everything else out

49
Q

Treatment of ITP

A

Observe in asymptomatic children

Avoid meds that compromise platelet function (ie ASA, NSAIDs)

Bleeding precautions

Prednisone (b/c it’s an autoimmune process)

IVIG

Splenectomy

50
Q

Most common inherited bleeding disorder

A

Von Willebrand Disease

Prevalence of 1%

Autosomal dominant, M=F

Decrease in the level of impairment in the action of von Willebrand Factor (vWF)

Divided into 3 major types but Type 1 most common

51
Q

A protein that binds to factor VIII and is a cofactor for platelet adhesion to the endothelium

A

von Willebrand factor

W/o it, platelets don’t clump and can’t get started

52
Q

Clinical presentation of von Willebrand disease

A

Prolonged bleeding from mucosal surfaces (epistaxis, menorrhagia, GI)

Easy bruising

53
Q

Lab findings for von Willebrand disease

A

Normal PT

Prolonged or normal aPTT

Normal or decreased Factor VIII

Normal or decreased vWF

PROLONGED bleeding time (helps differentiate from Hemophilia)

54
Q

Test that most helps differentiate VWD from Hemophilia

A

Bleeding time

55
Q

Treatment for von WIllebrand disease

A

Desmospressin (DDAVP) - causes release of vWF and factor VIII from endothelial stores

vWF-replacement therapy

56
Q

Most common form of Hemophilia

A

Hemophilia A (Factor VIII deficiency)

Hemophilia B is less common (Factor IX deficiency, aka Christmas disease)

Both are X-linked, M>F

Disease severity correlates with factor levels

57
Q

Clinical presentation of Hemophilia

A

Bleeding from impaired hemostasis

Varies with severity:
Mild disease:
• Bleed in response to injury/trauma or surgery
• May not become clinically apparent until later in life

Severe disease:
• Severe bleeding
• Spontaneous bleeding
• Earlier age of first bleeding episode

Onset of bleeding may be delayed by several days
Can occur anywhere —> common sites are INTO joints/muscles

58
Q

Spontaneous hemarthrosis is common in severe cases of

A

Hemophilia

If recurrent, can lead to joint destruction

59
Q

Lab findings in hemophilia patients

A

Normal platelet count, PT, and bleeding time

Prolonged aPTT b/c factor VIII on intrinsic side - can be normal in mild disease

Normal vWF

60
Q

Treatment for hemophilia

A

Desmopressin (DDAVP) if Hemophilia A

Factor replacement (VIII and IX) to achieve sufficient hemostasis

61
Q

Acquired syndrome characterized by hemorrhage and microvascular thrombosis

A

Disseminated Intravascular Coagulation (DIC)

Triggered by an event:
Sepsis
Trauma and tissue injury
Malignancies

62
Q

SSx of DIC

A

Shock —> end-organ dysfunction

Diffuse bleeding tendency
• Hematuria, melena, purpura, petechiae
• Persistent oozing from needle punctures or other invasive procedures

Evidence of thrombotic lesions
• Major vessel thrombosis
• Purpura fulminans

63
Q

Lab findings in DIC

A

Decreased platelet count

Prolonged aPTT and PT

Decreased fibrinogen level (may be normal until late in the course with children

Elevated D-Dimer and fibrin degradation products (FDPs)

64
Q

Treatment of DIC

A

Identification and treatment of the triggering event

Replacement therapy for consumptive coagulopathy

Anticoagulant therapy when indicated

65
Q

Liver is the major synthetic site of …

A

Prothrombin, fibrinogen

Factors V, VII, IX, X, XII, and XIII

66
Q

Which clotting factors are Vitamin K dependent?

A

II, VII, IX, and X

During the newborn period - physiologically depressed activity of the vitamin K dependent factors so must supplement

67
Q

Laboratory findings in vitamin K deficiency

A

Platelet count
• Normal in vitamin K deficiency
• Normal or low in liver disease

Prolonged PT, aPTT

68
Q

Treatment for vitamin K deficiency

A

Treat underlying condition

Vitamin K at birth

69
Q

Inherited thrombotic disorder that can lead to Warfarin-induced skin necrosis

A

Protein C deficiency

Homozygous and heterozygous forms

Activated protein C inactivates activated factors V and VIII

70
Q

Tell me about Protein S Deficiency

A

Homozygous and heterozygous forms

Protein S is a cofactor for Protein C

Facilitates the action of activated Protein C

71
Q

Risk of incident VTE is increased 2-7x in patients with…

A

Factor V Leiden Mutation

Point mutation —> Factor V polymorphism —> resistant to inactivation by activated protein C

Both heterozygous and homozygous

VTE risk 35-fold increased among heterozygous taking oral contraceptives

VTE risk 80-fold in those homozygous for factor V Leiden

72
Q

Tell me about antithrombin deficiency

A

Antithrombin is a major physiologic inhibitor of thrombin

Clinical presentation —> VTE

Efficiency of heparin may be significantly diminished

73
Q

Summary of thrombotic disorders

A

Hypercoagulable

Increased risk of thrombosis

May see DVTs or PEs

Clinical risk factors

Positive family history of thrombosis (always ask)

74
Q

Treatment of thrombotic disorders

A

Anticoagulant prophylaxis

Anticoagulant agents (UFH, LMWH, Warfarin)

Current guidelines largely based on adult experience (first episode of VTE, anticoagulation for at least 3 months)

May need long-term anticoagulation

75
Q

Most common type of small vessel vasculitis, primarily affecting boys aged 2-7 years

A

Henoch-Schonlein Purpura (HSP)

Occurrence highest in spring and fall

URI often precedes diagnosis

Deposition of IgA immune complexes in small vessels of the skin, GI tract, and kidneys

76
Q

Clinical presentation of Henoch-Schonlein Purpura

A

Palpable purpura

Arthritis/arthralgias

Abdominal pain

Renal disease

77
Q

Lab findings for HSP

A

Normal or elevated platelet count

Antistreptolysin O (ASO) titer is often elevated

Serum IgA may be elevated

Hemoccult may be positive

Urinalysis: hematuria, sometimes proteinuria

78
Q

Treatment and prognosis for HSP

A

Supportive care

Prognosis generally good

Peds II Unit I (4 decks)

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