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Hematology Flashcards

0
Q

Relationship between hemoglobin and age?

A
  1. High at birth
  2. lowest point at 2-3 months of age in term infant (1-2 months of age in preterm infants)
  3. Adult levels after puberty
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1
Q

Definition of anemia?

A

Reduction in red blood cells more than two standard deviations below the mean

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2
Q

When does fetal hemoglobin decline?

A

6-9 months of age

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3
Q

Most common blood disease during infancy? Age-related causes? Clinical features? Laboratory findings? Management?

A

Iron deficiency anemia

  1. 9-24 months of age - Inadequate intake/storage (Iron stores depleted by 6-9 months of age)
  2. Toddlers– Cows milk
  3. Adolescent girls – poor diet, growth, menstrual blood
  4. Any age: Occult blood loss – Meckel’s diverticulum, IBD, peptic also disease, early cows milk

Underweight, spoon shaped nails, diminished attention/ability to learn

Low ferritin, increased transferrin, decreased transferrin saturation

  1. 4-6 mg/kg/day iron given with vitamin C (Orange juice) to enhance absorption
  2. RBC transfusion if in congestive heart failure
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4
Q

Thalassemia – Pathophys of physical exam findings?

A

Increased bone marrow activity leads to increased marrow space results in increased size of bones face and skull

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5
Q

alpha-thalassemia – Ethnic group? Types?

A

Southeast Asians

  1. silent
  2. mild anemia
  3. Hemoglobin H disease – severe anemia with elevated hemoglobin Bart
  4. Only hemoglobin Barts – hydrous fetalis
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6
Q

Beta thalassemia major – ethnic group? Clinical features? Laboratory findings? Management? Complications?

A

Mediterranean

  1. Hepatosplenomegaly
  2. Bone marrow hyperplasia leading to frontal bossing, prominent cheekbones, skull deformities
  3. Hypochromia and Microcytosis with Target cells
  4. Elevated Bilirubin, serum iron, LDH
  5. Electrophoresis – absent hemoglobin A and elevated hemoglobin F

Lifelong transfusions, splenectomy, bone marrow transplant

Hemachromatosis from transfusions

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7
Q

Beta thalassemia minor – laboratory findings? Treatment?

A
  1. Hemoglobin levels 2 to 3 below norm
  2. Hypochromia with microcytosis, and target cells

No treatment

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8
Q

Causes of sideroblastic anemia?

A

Inherited

Isoniazid, alcohol, lead, chloramphenicol

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9
Q

Folic acid deficiency – causes in children? Clinical features? Diagnosis? Management?

A
  1. Diet without fruits/vegetables
  2. Exclusive feedings with goats milk
  3. Decreased absorption (celiac disease, enteritis, Crohn’s disease, anticonvulsants, OCP)

Failure to thrive, chronic diarrhea

High levels of homocysteine without increased methylmalonic acid

Dietary folic acid

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10
Q

B12 deficiency – causes and children? Clinical features? The diagnosis? Management?

A
  1. Inadequate dietary intake (Vegan diets)
  2. Juvenile pernicious anemia
  3. Malabsorption (Crohn’s disease)
  4. Smooth red tongue
  5. Neurologic – ataxia, hyporeflexia, positive Babinski responses

High homocystine and Methylmalonic acid

Monthly B12 injections

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11
Q

Child with normocytic anemia – causes of low reticulocyte count? High reticulocyte count?

A

Res cell aplasias, pancytopenia, malignancy

Hemolytic anemias, sickle cell

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12
Q

Most common inherited abnormality of red blood cell membrane? Inheritance? Mechanism? Clinical features? Lab findings? Management?

A

Hereditary spherocytosis; Ensemble dominant; defective spectrin

  1. Splenomegaly by three years of age
  2. Pigmented gallstones
  3. Aplastic crises with parvovirus infection
  4. Infants present with jaundice
  5. Elevated reticulocyte count
  6. hyperbilirubinemia
  7. spherocytes on smear
  8. abnormal osmotic fragility studies

Cured by splenectomy (usually done after five years of age)

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13
Q

Hereditary elliptocytosis – inheritance? Clinical findings? Treatment?

A

Autosomal dominant

  1. Most patients are asymptomatic
  2. Jaundice, splenomegaly, gallstones

Splenectomy only if patients have hemolysis

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14
Q

Pyruvate kinase deficiency – mechanism? Clinical features? Laboratory findings? Diagnosis? Management?

A

Decreased pyruvic kinases leading to ATP depletion and decreased red blood cell survival

Power, jaundice, splenomegaly – kernicterus in neonates.

Polychromatic red blood cells on smear

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15
Q

Most common red blood cell and somatic defect? Mechanism? Triggers? Clinical features? Laboratory findings? Diagnosis? Treatment?

A

G6PD deficiency – increased oxidative damage

Infection, fava beans, sulfa drugs, salicylates, antimalarials,

Jaundice, vomiting/diarrhea, fever

Bite cells and Heinz bodies on smear

Low G6PD in red blood cells

Transfusions

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16
Q

Fulminant versus prolonged autoimmune hemolytic anemia - course and prognosis? Laboratory findings? Management?

A

Occurs in children after respiratory infections (complete recovery expected) versus due to underlying cause (protracted course and high mortality)

Positive direct Coombs test

Corticosteroids

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17
Q

Alloimmune hemolytic anemia? Types?

A

Rh & ABO hemolytic diseases

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18
Q

Rh hemolytic disease? Test? Management?

A

Rh negative mother produces antibodies to Rh antigen in fetus. In subsequent pregnancies antibodies cause Mollasses in fetal red blood cells leading to kernicterus, anemia, hepatosplenomegaly, and hydrops fetalis

Strongly positive Coombs’ test

Phototherapy if mild jaundice, exchange transfusion it’s severe jaundice

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19
Q

ABO hemolytic disease? Test? Management?

A

O type mother produces antibodies to A/B blood group antigen causing hemolysis in fetus (can occur in the first pregnancy, unlike Rh disease)

Weekly positive direct Coombs’ test

Phototherapy for mild jaundice; exchange transfusion for severe jaundice

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20
Q

Sickle cell disease – mutation?

A

Valine for glutamic acid in sixth position of beta-globin chain

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21
Q

Findings in sickle cell trait patients? Symptoms?

A

Small percentage of hemoglobin S

  1. Asymptomatic without anemia unless exposed to severe hypoxemia
  2. During adolescence some patients have hematuria or are unable to concentrate urine
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22
Q

Most common type of crisis and sickle cell patients? Subtype? Duration? Management?

A

Painful bone crisis due to ischemia/infarction of bone

Subtype: acute dactylitis – swelling of digits

Last 3-7 days

  1. IV fluids at two times maintenance
  2. Incentive spirometry
  3. Pain control
  4. If unremitting pain – consider exchange transfusion (Regular transfusion may worsen crisis)
23
Q

Patient with sickle cell who presents with dysarthria, hemiplegia? Treatment?

A

Stroke

  1. Same management as for bone crisis but with urgent exchange transfusion
  2. chronic transfusions program to prevent recurrence
24
Always consider SS disease in any patient presenting with?
Priapism
25
Acute chest syndrome? Causes? Treatment?
Pulmonary infiltrate associated with respiratory symptoms Viral, bacterial, Sickling, fat embolism, fluid overload 1. Careful hydration and pain management 2. Cefuroxamine and azithromycin 3. Incentive spirometry 4. Early partial exchange transfusion if no rapid improvement
26
Sequestration crisis? Typical age? Physical exam findings? Management?
Rapid accumulation of blood and spleen liver Less than six years of age Abdominal distention/pain SOB Tachycardia Transfusion, splenectomy
27
Leading cause of death in sickle-cell? Management of fever in any patient with sickle cell?
Infection by encapsulated bacteria Cultures, CXR, parenteral antibiotics until infection ruled out
28
May mimic painful bone crisis? Most commonly caused by?
Osteomyelitis; salmonella
29
Lab findings in sickle cell anemia: 1. Red blood cell lifespan 2. Hemoglobin 3. Reticulocyte count 4. WBC count 5. Platelet count 6. Blood smear
1. 10 to 50 days 2. 6 to 9 3. 5-15% 4. 12,000 to 20,000 5. Greater than 500,000 6. Sickle cells, target cells, Howell-Jolly bodies
30
Preventative care for sickle cell?
1. Hydroxyurea (increases hemoglobin F) 2. Daily oral penicillin prophylaxis for first few months of life 3. Daily folic acid 4. Routine immunizations 5. Transcranial Doppler ultrasound or MRA beginning at age 2
31
Long-term complications of sickle cell?
1. Delayed growth/puberty 2. Cardiomegaly, cor pulmonale 3. Hemachromatosis 4. Gallstones 5. Poor wound healing 6. Avascular necklaces
32
Causes of red blood cells aplasias in childhood?
1. Parvovirus 2. Congenital hypoplastic anemia (Diamond-Blackfan anemia) 3. Transient erythroblastopenia of childhood
33
Fanconi anemia AKA? inheritance? Age of onset? Clinical features? Laboratory findings? Management?
Congenital aplastic anemia; autosomal recessive; seven years 1. Skeletal – short stature, absence of thumb/radius 2. Skin hyperpigmentation 3. Renal abnormalities Pancytopenia, RBC macrocytosis, low reticulocyte count, elevated hemoglobin F Transfusions, bone marrow transplant, immunosuppressive therapy
34
Congenital hypoplastic anemia a.k.a.? Age of onset? Clinical features? Lab findings? Treatment?
Diamond-Blackfan anemia; first year of life 1. Skeletal – short stature, triphalangeal thumbs 2. Renal/cardiac abnormalities Decreased hemoglobin, reticulocytes, platelet count, RBC precursors Transfusions, corticosteroids, Bonow transplant
35
Transient erythroblastopenia of childhood – age of onset? clinical features? Laboratory findings? Treatment?
Slow onset beginning at one year Decreased hemoglobin, reticulocytes, red blood cell precursors Spontaneous recovery within seven weeks
36
Definition of polycythemia? Most common cause? Clinical signs? Complications?
Hematocrit >60 Cyanotic Congenital heart disease Ruddy facial complexion with normal sized liver/spleen Thrombosis, bleeding
37
1. PTT in von Willebrand disease? 2. Bleeding time in thrombocytopenia? 3. Petechia seen in these four disorders?
1. Prolonged 2. Prolonged 3. Thrombocytopenia, platelet function defect, vitamin K deficiency, DIC
38
Platelet factor 2 AKA? Factor 1?
Prothrombin; fibrinogen
39
Management of hemophilia? Mech?
DDAVP to release stored factor 8
40
Signs of von Willebrand disease? Management?
Epistaxes, menorrhagia, bruising, bleeding after dental extraction 1. DDAVP to release von Willebrand factor from endothelial cells 2. Cryoprecipitate
41
Causes of vitamin K deficiency?
1. Pancreatic insufficiency, biliary obstruction 2. Prolonged diarrhea 3. Medications (of cephalosporins, rifampin, warfarin, isoniazid)
42
Congenital disorders with decreased platelet production (And other findings)?
Wiskott-Aldrich syndrome – Small platelets, eczema, defects in T/B cell immunity Thrombocytopenic Absent-radius syndrome – No radius (but unlike Fanconi, thumb is present), ASD/tetralogy, renal disease
43
ITP – when to treat?
Platelet count less than 20,000 – IVIG, corticosteroids, anti-D immunoglobulin (if Rh positive)
44
Passive autoimmune thrombocytopenia versus Isoimmune thrombocytopenia?
ITP antibodies destroy fetus' platelets. Mother HAS thrombocytopenia Mother produces antibodies against fetus' platelets. Mother does NOT have thrombocytopenia
45
Kasabach-Merritt Syndrome - pathophysiology? Characteristics?
Large hemangiomas sequester/destroy platelets hemangioma, microangiopathic hemolytic anemia, thrombocytopenia
46
Glanzmann's versus Bernard-Soulier syndrome
Decreased platelet aggregation (glycoprotein IIb/IIIa versus decreased platelet adhesion
47
Inherited hypercoagulabilities?
1. Protein C/S deficiency 2. Antithrombin III deficiency 3. Factor five Leiden deficiency
48
Protein C deficiency – initially presents with (specific name)? Clinical features? And itching?
Purpura Fulminans (non-thrombocytopenic purpura) Fever, shock, skin bleeding, thrombosis Heparin, FFP, warfarin
49
Mild versus moderate versus severe neutropenia?
Absolute neutrophil count is: 1000-1500 versus 500-1000 versus <500
50
Common cause of neutropenia in children younger than four? Clinical features? Laboratory findings? Prognosis?
Chronic benign neutropenia of childhood Increased incidence of mild infections (otitis media, sinusitis, pharyngitis, cellulitis) 1. Low absolute neutrophil count 2. immature neutrophil precursors in bone marrow Resolve spontaneously
51
Kostmann Syndrome – a.k.a.? Absolute neutrophil count? Clinical features?
Severe congenital agranulocytosis; <300 Frequent pyogenic bacterial infections beginning in infancy
52
Cyclic neutropenia – Pathophysiology? clinical features? Diagnosis?
Cyclical alterations and nutrition counseling result in episodes of neutropenia with infections Fever, oral ulcers, stomatitis Serial neutrophil counts during 2 to 3 months.
53
Chediak-Higashi – clinical features? Histo?
1. Albinism 2. Blonde hair with silver streaks 3. Infections Blue-great granules in cytoplasm of neutrophils
54
Cartilage-hair hypoplasia syndrome – characterized by?
1. Short stature 2. Immunodeficiency/neutropenia 3. Fine hair
55
Schwachman-Diamond syndrome – characterized by?
1. Short stature due to metaphyseal chondrodysplasia 2. Neutropenia 3. Exocrine pancreatic insufficiency with Malabsorption
56
Metabolic diseases that can result in neutropenia?
Hyperglycemia, methylmalonic acidemia, Gaucher's disease

Pediatrics (38 decks)

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