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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
Lab findings in megaloblastic anemia
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
26
Treatment for megaloblastic anemia
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
27
Red cell membrane defect leading to hemolytic anemia —> jaundice, splenomegaly, gallstones
Hereditary Spherocytosis (HS) Increased osmotic fragility —> spherocytes Tx: supportive care +/- RBC transfusion, may need a splenectomy (make sure they have pneumococcal vaccine first
28
Severity of Thalassemia is related to...
The number of gene deletions Can affect either Alpha or Beta chains of hemoglobin
29
Microcytic hypochromic anemia of variable severity, due to genetic deletions of alpha or beta chain genes
Thalassemia Dx via hemoglobin electrophoresis Supportive +/- RBC transfusion • Iron monitoring and chelation • Splenectomy may help • HSCT if severe beta-thalassemia
30
HbS electrophoresis is used to diagnose...
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
31
Treatment options for Sickle Cell disease
``` Avoiding precipitating factors Supportive care Hydroxyurea Treatment for painful vaso-occlusive episodes HSCT ```
32
X-linked recessive red cell enzyme defect that causes hemolytic anemia
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
Clinical manifestations of G6PD
Neonatal jaundice, hyperbilirubinemia Episodic hemolysis (pallor, jaundice, hemoglobinuria)
34
Laboratory findings for G6PD
Bite-like deformities and Heinz bodies (denatured hemoglobin) on peripheral smear
35
Treatment for G6PD
Supportive treatment Avoidance of certain foods and drugs known to trigger hemolysis
36
Basophilia stippling (hehehe)
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
Primary polycythemia is also referred to as...
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
Secondary polycythemia occurs in response to ...
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
Initial screening tests for bleeding disorders
CBC (specifically, platelets) Peripheral smear PT/INR, aPTT +/- bleeding time
40
What is a normal platelet count?
150,000-400,000/mm3 (varies little with age) Risk of spontaneous bleeding if <20,000
41
What does the prothrombin time (PT) test measure?
Measures EXTRINSIC and common pathways I, II, V, VII, X, and Tissue Factor
42
What does the activated partial thromboplastin time (PTT/aPTT) test measure?
Measures INTRINSIC and common pathways (think, it puts another T IN it) I, II, V, VIII, IX, X, XI, XII
43
INR is a more accurate reflection of ...
PT Used to monitor Warfarin treatment
44
Bleeding time test is a measurement of ...
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
Most common bleeding disorder of childhood
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
What is the pathophysiology of ITP?
Immune mediated attach against one’s own platelets Autoantibodies bind to platelets —> Platelets are phagocytized primarily by splenic macrophages —> Decreased platelet life span
47
Clinical manifestations of ITP
Multiple petechiae Ecchymosis Epistaxis ``` Labs: Thrombocytopenia Normal WBC Normal Hb (unless hemorrhage) PT and aPTT are normal (ALL tests are normal except platelets) ```
48
Dx of ITP is ...
A diagnosis of exclusion - rule everything else out
49
Treatment of ITP
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
Most common inherited bleeding disorder
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
A protein that binds to factor VIII and is a cofactor for platelet adhesion to the endothelium
von Willebrand factor W/o it, platelets don’t clump and can’t get started
52
Clinical presentation of von Willebrand disease
Prolonged bleeding from mucosal surfaces (epistaxis, menorrhagia, GI) Easy bruising
53
Lab findings for von Willebrand disease
Normal PT Prolonged or normal aPTT Normal or decreased Factor VIII Normal or decreased vWF PROLONGED bleeding time (helps differentiate from Hemophilia)
54
Test that most helps differentiate VWD from Hemophilia
Bleeding time
55
Treatment for von WIllebrand disease
Desmospressin (DDAVP) - causes release of vWF and factor VIII from endothelial stores vWF-replacement therapy
56
Most common form of Hemophilia
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
Clinical presentation of Hemophilia
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
Spontaneous hemarthrosis is common in severe cases of
Hemophilia If recurrent, can lead to joint destruction
59
Lab findings in hemophilia patients
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
Treatment for hemophilia
Desmopressin (DDAVP) if Hemophilia A Factor replacement (VIII and IX) to achieve sufficient hemostasis
61
Acquired syndrome characterized by hemorrhage and microvascular thrombosis
Disseminated Intravascular Coagulation (DIC) Triggered by an event: Sepsis Trauma and tissue injury Malignancies
62
SSx of DIC
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
Lab findings in DIC
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
Treatment of DIC
Identification and treatment of the triggering event Replacement therapy for consumptive coagulopathy Anticoagulant therapy when indicated
65
Liver is the major synthetic site of ...
Prothrombin, fibrinogen | Factors V, VII, IX, X, XII, and XIII
66
Which clotting factors are Vitamin K dependent?
II, VII, IX, and X During the newborn period - physiologically depressed activity of the vitamin K dependent factors so must supplement
67
Laboratory findings in vitamin K deficiency
Platelet count • Normal in vitamin K deficiency • Normal or low in liver disease Prolonged PT, aPTT
68
Treatment for vitamin K deficiency
Treat underlying condition Vitamin K at birth
69
Inherited thrombotic disorder that can lead to Warfarin-induced skin necrosis
Protein C deficiency Homozygous and heterozygous forms Activated protein C inactivates activated factors V and VIII
70
Tell me about Protein S Deficiency
Homozygous and heterozygous forms Protein S is a cofactor for Protein C Facilitates the action of activated Protein C
71
Risk of incident VTE is increased 2-7x in patients with...
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
Tell me about antithrombin deficiency
Antithrombin is a major physiologic inhibitor of thrombin Clinical presentation —> VTE Efficiency of heparin may be significantly diminished
73
Summary of thrombotic disorders
Hypercoagulable Increased risk of thrombosis May see DVTs or PEs Clinical risk factors Positive family history of thrombosis (always ask)
74
Treatment of thrombotic disorders
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
Most common type of small vessel vasculitis, primarily affecting boys aged 2-7 years
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
Clinical presentation of Henoch-Schonlein Purpura
Palpable purpura Arthritis/arthralgias Abdominal pain Renal disease
77
Lab findings for HSP
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
Treatment and prognosis for HSP
Supportive care Prognosis generally good

Peds II Unit I (4 decks)

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