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Pediatrics > Hematology > Flashcards

Hematology Flashcards

1
Q

Most common laboratory abnormality during childhood

A

Anemia

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2
Q

Microcytic, hypochromic anemias

A
  • Iron deficiency
  • Thalassemia
  • Sideroblastic anemia
  • Lead toxicity
  • Chronic disease
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3
Q

Macrocytic anemias

A
  • Vitamin B12 deficiency
  • Thiamine deficiency
  • Folate deficiency
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4
Q

Normocytic, normochromic anemias with high reticulocyte count

A
  • Hemangioma
  • DIC
  • Hemolytic anemias
  • Sickle cell disease
  • Blood loss
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5
Q

Normocytic, normochromic anemias with low reticulocyte count

A
  • Red cell aplasia
  • Malignancy
  • Fanconi anemia
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6
Q

Hemolytic anemias due to intrinsic RBC defects

A
  • RBC membrane disorders: hereditary spherocytosis, hereditary elliptocytosis
  • RBC enzyme disorders: G6PD deficiency, pyruvate kinase deficiency
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7
Q

Hemolytic anemias due to extrinsic RBC defects

A
  • HUS
  • Autoimmune
  • Alloimmune
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8
Q

Red cell aplasias

A
  • TEC
  • Diamond-Blackfan syndrome
  • Parvovirus B19 infection
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9
Q

Occult blood loss with resultant iron deficiency may be secondary to

A
  • Polyps
  • Meckel’s diverticulum
  • Inflammatory bowel disease
  • Peptic ulcer disease
  • Early ingestion of whole cow’s milk before 1 year of age
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10
Q

Clinical features of beta thalassemia major

A
  • Hemolytic anemia
  • Hepatosplenomegaly
  • Bone marrow hyperplasia in sites that results in a characteristic thalassemia facies (frontal bossing, maxillary hyperplasia with prominent cheekbones and skill deformities)
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11
Q

What can beta thalassemia minor be easily misdiagnosed as

A

Iron deficiency anemia, however iron level is normal or elevated in these patients

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12
Q

Sideroblastic anemia

A
  • Ringed sideroblasts in the bone marrow
  • Result from the accumulation of iron in the mitochondria of RBC precursors
  • Acquired as a result of drugs or toxins (isoniazid, alcohol, lead poisoning, chloramphenicol)
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13
Q

Clinical features of vitamin B12 deficiency

A
  • Anorexia
  • Smooth red tongue
  • Neurologic manifestations (ataxia, hyporeflexia, positive Babinksi)
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14
Q

Clinical features of hereditary spherocytosis

A
  • Splenomegaly by 2-3 years of age
  • Pallor
  • Weakness
  • Pigmentary gallstones
  • Aplastic crises (most commonly associated with parvovirus B19)
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15
Q

Clinical features of hereditary elliptocytosis

A
  • Most patients asymptomatic
  • 10% have jaundice at birth
  • Splenomegaly
  • Gallstones
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16
Q

Inheritance pattern of hereditary spherocytosis and hereditary elliptocytosis

A

Autosomal dominant

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17
Q

Pyruvate kinase deficiency

A
  • Autosomal recessive

- Decreased production of PK isoenzyme leading to ATP depletion and decreased RBC survival

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18
Q

Clinical features of pyruvate kinase deficiency

A
  • Pallor
  • Jaundice
  • Splenomegaly
  • Kernicterus in neonates
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19
Q

Laboratory findings of pyruvate kinase deficiency

A
  • Varying degrees of anemia

- Blood smear showing polychromatic RBCs

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20
Q

Most common RBC enzymatic defect

A

Glucose-6-phosphate dehydrogenase deficiency

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21
Q

Types of autoimmune hemolytic anemia

A
  • Primary: generally idiopathic; viral infections and occasionally drugs
  • Secondary: underlying disease process, such as lymphoma, SLE, immunodeficiency disease
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22
Q

Fulminant acute type AIHA

A
  • Infants and young children
  • Preceeded by a respiratory infection
  • Acute onset of pallor, jaundice, hemoglobinuria and splenomegaly
  • Complete recovery is expected
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23
Q

Prolonged type AIHA

A
  • Protracted and high mortality

- Underlying disease is frequently present

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24
Q

Types of alloimmune hemolytic anemias

A
  • Rh hemolytic disease

- ABO hemolytic disease

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25
Q

Etiology of microangiopathic hemolytic anemia

A
  • Severe hypertension
  • Hemolytic uremic syndrome
  • Artificial heart valves
  • Giant hemangioma
  • Disseminated intravascular coagulation
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26
Q

Leading cause of death in patients with sickle cell disease

A

Infection - up to 30% of patients develop sepsis or meningitis in the first 5 years of life

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27
Q

Preventative care in sickle cell disease

A
  • Hydroxyurea - decreases incidence of vasoocclusive crises
  • Daily oral penicillin prophylaxis is started in the first few months of life to decrease the risk of S pneumonia infection
  • Daily folic acid is given to prevent deficiency
  • Routine immunizations
  • Serial transcranial Doppler ultrasound or magnetic resonance angiography is recommended beginning at 2 years to identify patients at risk for stroke
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28
Q

Long term complications of sickle cell disease

A
  • Delayed growth and puberty
  • Cardiomegaly
  • Hemochromatosis
  • Cor pulmonale
  • Gallstones
  • Poor wound healing
  • Avascular necrosis of the femoral and humeral heads
  • Diminished cognition and school performance
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29
Q

Pancytopenia

A

Bone marrow failure with decreased RBCs, leukocytes and platelets

30
Q

Congenital aplastic anemia/ Fanconi anemia

A
  • Autosomal recessive
  • Onset of bone marrow failure occurs at mean age of 7 years
  • Typical presentation is with ecchymosis and petechiae
  • Skeletal abnormalities, which include short stature is almost all patients, and absence or hypoplasia or the thumb and radius
  • Skin hyper pigmentation
  • Renal abnormalities
31
Q

Laboratory findings of Fanconi anemia

A
  • Pancytopenia
  • RBC macrocytosis
  • Low reticulocyte count
  • Elevated HbF
  • Bone marrow hypocellularity
32
Q

Etiologies of acquired aplastic anemia

A
  • Drugs: sulfonamides, anticonvulsants, chloramphenicol
  • Infections: HIV, EBV, CMV
  • Chemicals
  • Radiation
  • Idiopathic
33
Q

Appropriate polycythemia may be caused by chromic hypoxemia as a result of

A
  • Cyanotic congenital heart disease (most common cause of polycythemia in childhood)
  • Pulmonary disease
  • Residence at high altitudes
34
Q

Inappropriate polycythemia may be caused by

A
  • Benign and malignant tumors of the kidney, cerebellum, ovary, liver and adrenal glands
  • Excess hormone production (corticosteroids, growth hormone, androgens)
  • Kidney abnormalities (hydronephrosis)
35
Q

Clinical features of polycythemia

A

Ruddy facial complexion and normal sized liver and spleen

36
Q

Phlebotomy is used to keep the hematocrit under what in polycythemia

A

60%

37
Q

Relative polycythemia

A

Apparent increase in RBC mass caused by a decrease in plasma volume. Most commonly caused by dehydration.

38
Q

Describe severe, moderate and mild forms of Hemophilia A

A
  • SEVERE: spontaneous bleeding (<1% factor VIII protein activity)
  • MODERATE: bleeding only with trauma (1-5% factor VIII protein activity)
  • MILD: bleeding only after surgery or major trauma (>5% factor VIII protein activity)
39
Q

Describe types of von Willebrand’s disease

A
  • Type I: classic type, mild quantitative deficiency of vWf and factor VIII protein; most common form
  • Type II: qualitative abnormality in vWf
  • Type III: absence of vWf; most severe type
40
Q

Laboratory findings of von Willebrand’s disease

A
  • Prolonged BT and aPTT may be present, but not always (they are always present in type III disease)
  • Quantitative assay for vWf antigen and activity (ristocetin cofactor assay) are diagnostic
41
Q

Hemorrhagic disease of the newborn

A

Special type of vitamin K deficiency. It may occur EARLY (within 24 hours after birth), within the first week of life (CLASSIC form), or LATE (1-3 months after birth)

42
Q

Laboratory findings of liver disease

A
  • Same as those seen in DIC
  • Prolonged PT and aPTT
  • Increased fibrin degradation products
  • Thrombocytopenia
43
Q

Disorders of blood vessels

A
  • These diseases affect the integrity of blood vessels and may present with bleeding
  • Henoch-Schonlein purpura
  • Hereditary hemorrhagic telangiectasia
  • Scurvy
  • Inherited disorders of collagen synthesis
  • Malnutrition and corticosteroids
44
Q

Henoch-Schonlein purpura

A
  • IgA mediated vasculitis
  • Presents with palpable purport on the lower extremities and buttocks, renal insufficiency, arthritis and abdominal pain
  • Platelet count is normal
45
Q

Hereditary hemorrhagic telangiectasia

A
  • Autosomal dominant

- Characterized by locally dilated and tortuous veins and capillaries of the skin and mucous membranes

46
Q

Most common cause of bleeding

A

Thrombocytopenia

47
Q

Quantitative platelet disorders that involve decreased platelet production

A
  • Wiskott-Aldrich syndrome

- Thrombocytopenia-absent radius syndrome

48
Q

Quantitative platelet disorders that involve increased platelet destruction

A
  • Immune thrombocytopenic purpura
  • Neonatal immune-mediated thrombocytopenia
  • Drugs, DIC and enlarged spleen
  • Hemolytic uremic syndrome
  • Large hemangioma
49
Q

Qualitative platelet disorders

A
  • Glanzmann’s thrombasthenia
  • Bernard-Soulier syndrome
  • Drugs (aspirin, valproate), uremia, severe liver disease
50
Q

Wiskott-Aldrich syndrome

A
  • X linked
  • Thrombocytopenia with unusually small platelets
  • Eczema
  • Defects in T and B cell immunity
51
Q

Thrombocytopenia-absent radius syndrome

A
  • Autosomal recessive
  • Thrombocytopenia
  • Limb abnormalities (absence of radius, but THUMB is present)
  • Cardiac and renal disease
  • Thrombocytopenia improves in second or third year of life
52
Q

Most commonly acquired platelet abnormality in childhood

A

Immune thrombocytopenic purpura

53
Q

Etiology of ITP

A
  • Viral
  • Drug induced
  • Idiopathic
54
Q

Pathophysiology of ITP

A

Because ITP often follows a viral infection, it is thought that the virus triggers antibodies that cross react with platelets, causing their destruction and removal by the spleen

55
Q

Clinical features of ITP

A

Illness typically occurs 1-4 weeks after a viral infection. It begins abruptly with cutaneous bleeding (e.g. petechiae, bruising) or mucous membrane bleeding (e.g. epistaxis, gum bleeding). Internal bleeding into the brain (occurs in <1%), kidneys or GI tract may occur but is rare.

56
Q

Laboratory findings of ITP

A

Studies reveal thrombocytopenia and a blood smear showing few large “sticky” platelets

57
Q

Management of ITP

A
  • Supportive care
  • IVIG or corticosteroids for low platelets
  • 2nd line: Anti-D immunoglobulin - binds to erythrocyte D antigen on RBCs and allows them to be preferentially cleared by the spleen, allowing platelets to escape destruction
  • Platelet transfusions are generally avoided because transfused platelets are rapidly destroyed
58
Q

Passive autoimmune thrombocytopenia vs isoimmune thrombocytopenia of the neonate

A
  • PASSIVE - mother has ITP and antibodies against her own platelets cross the placenta and destroy the fetus’s platelets; mom has thrombocytopenia
  • ISOIMMUNE - occurs when mother produces antibodies against her fetus’s platelets as a result of sensitization to an antigen that her own platelets lack; mom does not have thrombocytopenia
59
Q

Kasabach-Merritt syndrome

A

Characterized by an enlarging hemangioma, microangiopathic hemolytic anemia, thrombocytopenia and consumptive coagulopathy

60
Q

Inherited coagulation abnormalities leading to hypercoagulability

A

Deficiencies of proteins C or S or antithrombin III or mutations in factor V (factor V Leiden)

61
Q

Protein C deficiency

A

A vitamin K dependent factor that is the most potent anticoagulant protein known. Inheritance may be either AR or AD.

62
Q

Clinical features of protein C deficiency

A
  • HOMOZYGOTES: no protein C activity and are detected soon afar birth; PURPURA FULMINANS (nonthrombocytopenic purport) is often the initial presentation; characterized by fever, shock and rapidly spreading skin bleeding and IV thrombosis
  • HETEROZYGOTES: present later with deep venous or CNS thrombosis
63
Q

Most common cause of neutropenia during childhood

A

Infections may all suppress the bone marrow, marginate neutrophils or exhaust marrow reserves resulting in neutropenia:

  • Viruses (HIV, CMV, EBV, hepatitis A and B, influenza A, parvovirus B19)
  • Bacteria (typhus, rocky mountain spotted fever)
  • Protozoan (malaria)
64
Q

Chronic benign neutropenia of childhood

A

Common cause of neutropenia in children younger than 4 years, refers to a group of acquired and inherited disorders with NONCYCLIC neutropenia as the only abnormality. In most, resolves spontaneously within months to years.

65
Q

Clinical features of chronic benign neutropenia of childhood

A
  • Variable course
  • Most children have increased incidence of mild infections (otitis media, sinusitis, pharyngitis, and cellulitis)
  • Severe infections may occur but are uncommon
  • Children are otherwise healthy with normal appearance and growth
66
Q

Laboratory findings of chronic benign neutropenia of childhood

A
  • Low ANC
  • Normal or slightly low WBC
  • Bone marrow demonstrates immature neutrophil precursors (development of mature neutrophils is arrested)
67
Q

Severe congenital agranulocytosis/ Kostmann syndrome

A

Autosomal recessive disorder with frequent and life threatening pyogenic bacterial infections beginning in infancy. ANC usually less than 300.

68
Q

Chediak-Higashi syndrome

A
  • Autosomal recessive
  • Oculocutaneous albinism
  • Large blue gray granules in the cytoplasm of neutrophils
  • Neutropenia
  • Blond or brown hair with silver streaks
  • Patients are at high risk for serious infection
69
Q

Cartilage-hair hypoplasia syndrome

A
  • Autosomal recessive
  • Short stature
  • Immunodeficiency
  • Fine hair
  • Neutropenia
70
Q

Schwachmann-Diamond syndrome

A
  • Exocrine pancreatic insufficiency with malabsorption
  • Short stature caused by metaphyseal chondrodysplasia
  • Neutropenia
  • Failure to thrive and recurrent infections (esp otitis media) are common

Pediatrics (11 decks)

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