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Pediatrics > Hematology > Flashcards

Hematology Flashcards

1
Q

Physiologic nadir of Hgb levels

A

Between 2-3 months of age

Preterm= 1-2 months of age

-Reaches adult levels after puberty

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2
Q

Most common blood disease during infancy and childhood

A

Iron deficiency anemia; usually caused by inadequate intake but also occasionally to occult blood loss

-Iron stores are depleted by 6-9months of age, therefore, they need iron-rich cereal

Signs/Symptoms:

  • Pallor
  • tachycardia, tachypnea, fatigue
  • CHF
  • spoon shaped nails
  • diminished attention and inability to learn
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3
Q

Lab findings of anemia

A

Early: Low serum ferritin

Later: Increased transferrin, decreased transferrin saturation

-Increased free erythrocytes protoporphyrin

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4
Q

A-thalassemia

A

Occurs predominantly in Southeast Asians; has four levels

  1. Silent carrier; one gene deleted
  2. A-thalassemia minor: mild anemia
  3. Hb H disease: severe anemia at birth with elevated Hgb Bart’s
  4. Hydrops fetalis
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5
Q

B-thalassemia (2 kinds)

A

Major: Total absence of B-globin prod.; ️Occurs predominantly in Mediterranean people

Signs/Symptoms: Hepatosplenomegaly, bone marrow hyperplasia ➡️frontal bossing, chipmunk cheeks, skull deformities)

Minor: Mild, asymptomatic anemia
⭐️may be confused with iron deficiency anemia, BUT, The iron levels will be NORMAL

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6
Q

B-thalassemia labs (2 types)

A 
Major:
   Microcytic, Hypochromic RBCs; 
   elevated unconj. Bilirubin, iron, and LDH
   Poikilocytosis
   Absent Hb A and elevated Hb F

Minor:
Microcytic, Hypochromic RBCs
Target cells
Anisocytosis

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7
Q

B-thalassemia Tx.

A

Chronic transfusions; sometimes splenectomy

⭐️Chronic transufsions➡️ HEMOCHROMATOSIS

-Use deferoxamine to prevent this complication

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8
Q

Sideroblastic anemia

A

Characterized by the presence of ringed sideroblasts in the marrow resulting from the accumulation of iron in the RBC precursors

-Causes: Inheritance, chloramphenicol, isoniazid, lead poisoning, alcohol

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9
Q

Folic acid deficiency

A

Causes a macrocytic, Megaloblastic anemia; presents as failure to thrive, chronic diarrhea, and other signs of anemia

Causes: GOATS MILK FEEDING, diet with no fruit or vegetables, and decreased absorption (IBD, celiac, anticonvulsants)

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10
Q

Causes of Vitamin B12 deficiency

A

Inadequate dietary intake due to strict vegan diet

Inability to secrete intrinsic factor from parietal cells (Pernicious anemia)

Inability to absorb B12 (Crohn’s)

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11
Q

B12 deficiency signs and management

A

Characteristic anemia signs

Smooth, red tongue

Ataxia, hyporeflexia, Babinski (+)

Tx: Monthly B12 injection

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12
Q

Most common inherited abnormality of RBC membrane

A

Hereditary Spherocytosis; AD disorder usually in Northern Europeans

-Due to spectrin deficiency

Signs/Symptoms:
   Splenomegaly
  Pigmented gallstones
  Aplastic crises w/ Parvo B19 infxn
   Pallor 
  Weakness

Lab: (+) Osmotic fragility test

Tx: Splenectomy after age 5

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13
Q

Hereditary elliptocytosis

A

AD disorder of spectrin; usually asymptomatic

Can Tx. W/ splenectomy IF symptomatic

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14
Q

PK deficiency

A

AR disorder resulting in ATP depletion an decreased RBC survival

Signs/Symptoms:
   Pallor
  jaundice
   Splenomegaly
   Possible kernicterus

Tx: Transfusions, splenectomy

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15
Q

G6PD deficiency (from table)

A

Occurs in Mediterranean and African people

Triggers for hemolysis are fava beans, sulfa drugs, antimalarials, and infxn
-️Occurs 24-48hrs after exposure

Lab findings: 
  Hemoglobinuria
   Increased reticulocytes count
   Bite cells
   Heinz bodies

Tx: Transfusions as needed

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16
Q

Fulminant Acute type AIHA

A

Acute onset of pallor, jaundice, hemoglobinuria, and splenomegaly in an infant w/ a history of RESPIRATORY INFECTION

-Complete recovery expected unlike prolonged type AIHA (due to chronic illness)

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17
Q

ABO hemolytic disease of the newborn

A

Mother has blood type O and fetus is any other blood type

=» Abs cross the placenta causing slight hemolysis

***DAT=WEAKLY positive

*Can occur in first pregnancy unlike Rh disease

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18
Q

SS trait

A

Having only one HbS substitution of Valine for Glutamic Acid at the #6 position of the B-globin chain

**Electrophoresis shows HbA (50-60%), HbS (35-40%), and small amnt of HbF

-Usually asymptomatic but possible hematuria in adolescence

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19
Q

Most common crisis w/ SCD

A

Painful bone crisis

-occurs due to infarction of bone marrow

Signs/Symptoms: Deep, throbbing pain lasting 3-7 dys

-Subtype=Acute dactylitis

DDx: OSTEOMYELITIS

Tx: IV pain control
2x MTX fluids

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20
Q

Abdominal pain and distention in SCD pt.

A

Acute abdominal crisis

-caused by sickling within the SMA

DDX: Cholecystitis, appendicitis, splenic sequestration

Tx: IV pain control
2x MTX fluids

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21
Q

Dysarthria, hemiplegia in an SCD pt.

A

Stroke

Tx: IV pain control
2x MTX fluids
**Urgent exchange transfusion
**Chronic transfusions to prevent reoccurrence

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22
Q

Painful, sustained erections

A

Priapism =»> SUSPECT SCD

Tx: IV pain control
2x MTX fluids

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23
Q

New pulmonary infiltrate w/ SOB, cough, and chest pain in SCD pt.

A

Acute chest syndrome

Causes up to 25% of deaths in SCD; caused by infxn, sickling, atelectasis, fat embolism

Tx: Careful hydration
O2
Azithromycin and Cefuroxime if indicated

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24
Q

Rapid accumulation of blood in the spleen causing abdominal distention in an SCD pt.

A

Sequestration crisis

-Occurs in pts.

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25
Q

Previous parvovirus B19 infxn in SCD pt.

A

Aplastic crisis

Signs of anemia

Labs: Decreased Hgb, decreased reticulocytes

Tx: Supportive
RBC transfusion

26
Q

Rapid hemolysis in an SCD pt.

A

Hyperhemolytic crisis; usually coexisting G6PD deficiency

Signs of anemia + jaundice

Labs: Increased reticulocytes, increased bilirubin, decreased Hgb

Tx: RBC transfusion

27
Q

SCD lab findings

A

RBC lifespan: 10-50 days

Hgb: 6-9g/dL

Hcrt: 18-27%

Retic count: 5-15%

Platelets: Increased

Bilirubin: Increased

Blood smear: ***Howell-Jolly Bodies, sickle cells

28
Q

Osteomyelitis in SCD

A

Caused by Salmonella; mimics acute bone crisis

29
Q

Preventative care in SCD

A
  1. Hydroxyurea =» Increased HbF =» decreased occlusive crises
  2. Oral penicillin in first few months =» decreased S. pneumoniae
  3. Daily folic acid
  4. Routine vaccinations
  5. Transcranial US to evaluate stroke risk
30
Q

Congenital hypoplastic anemia

A

Diamond-Blackfan Syndrome

Signs: 
   Anemia in first year of life
   Craniofacial, cardiac, and renal abnormalities 
   Short stature 
   ***Triphalangeal thumbs

Labs:
Decreased- Hgb, retics, and RBC precursors in BM
Increased- Hgb F

Tx: RBC transfusion
Corticosteroids
BM transplant = last resort

31
Q

Postviral autoimmune rxn causing red blood cell aplasia

A

Transient erythroblastopenia of childhood

Signs:
Slow onset
Anemia signs and symptoms

Labs:
Decreased-Hgb, retics, RBC precursors in BM

Tx: Spontaneous recovery

32
Q

Congenital Aplastic Anemia

A

Fanconi Anemia (AR inheritance); begins at 7yrs of age

Signs:
   Short stature 
   Hypoplasia of thumb and radius 
   Hyperpigmentation
   Renal probs

Labs: BM hypocellularity, pancytopenia, macrocytosis

Tx: RBC transfusions
BM transplant if possible

33
Q

Primary polycythemia

A

Polycythemia vera

  • Malignancy involving RBC precursors
  • Very rare in childhood
34
Q

Most common cause of polycythemia in childhood

A

Cyanotic congenital heart disease

-Causes secondary polycythemia

Signs: Ruddy complexion

LabS: Increased Hb and Hcrt, normal platelets and WBCs
Increased EPO

-Can also be due to pulmonary disease or living at high altitude

35
Q

Most common cause of relative polycythemia

A

Dehydration

36
Q

Lab findings in Classic hemophilia

A

aPTT: Prolonged

PTT: Normal

Bleeding time: Normal

Platelet count: Normal

Petechiae: No

Hemarthroses: Yes

37
Q

Labs in vWF disease

A

aPTT: Prolonged

PTT: Normal

Bleeding time: Prolonged

Platelet count: Normal

Petechiae: No

Hemarthroses: No

38
Q

Labs in thrombocytopenia

A

aPTT: Normal

PTT: Normal

Bleeding time: Prolonged

Platelet count: Low

Petechiae: Yes

Hemarthroses: N

39
Q

Platelet fnxn defect labs

A

aPTT: Normal

PTT: Normal

Bleeding time: Prolonged

Platelet count: Normal

Petechiae: Yes

Hemarthroses: Yes

40
Q

Vitamin K deficiency labs

A

aPTT: Prolonged

PT: Prolonged

Bleeding time: Normal

Platelet count: Normal

Petechiae: Yes

Hemarthroses: Yes

41
Q

DIC labs

A

aPTT: Prolonged

PTT: Prolonged

Bleeding time: Prolonged

Platelet count: Low

Petechiae: Yes

Hemarthroses: Sometimes

42
Q

Hemophilia A

A

Factor VIII deficiency; XLR inherited disorder

Signs:
Hemarthroses
Deep tissue bleeding (especially into iliopsoas)

Mild has >5% actibity

Tx: Desmopressin acetate

43
Q

Most common hereditary bleeding disorder

A

vWF disease; AD inherited disorder

Type I: Quantitative (MOST COMMON)
Type II: Qualitative
Type III: Completely absent

Signs:
Menorrhagia
Epistaxis
Bleeding after dental procedures

Tx: Desmopressin acetate
Cryoprecipitate for serious bleeding and surgery

44
Q

Vitamin K deficiency causes

A

Pancreatic insufficiency,
biliary obstruction,
diarrhea,

rifampin,
isoniazid,
warfarin,

possible dietary right after birth

45
Q

Serious bleeding right after birth

A

Hemorrhagic disease of the newborn; due to Vitamin K deficiency

Tx: IM Vitamin K

-FFP may be need to tx if it is currently happening

46
Q

Locally dilated and torturous veins of the skin and mucous membranes

A

Hereditary hemorrhagic telangectasia

47
Q

Most common cause of bleeding

A

Thrombocytopenia

48
Q

Thrombocytopenia-absent radius syndrome (is pretty self-explanatory)

A

(TAR)

-AR disorder

Signs/Symptoms:
Thrombocytopenia (improves by Year 2)
Absence of the radius BUT THE THUMB IS THERE
Possible cardiac and renal disease

49
Q

Most common acquired platelet abnormality in childhood

A

ITP ; typically occurs 1-4 weeks after viral illness beginning w/ cutaneous or mucous membrane bleed

Labs: **Large, sticky platelets

Tx: IVIG
-Second line= Rhogam

-Typically resolves spontaneously but can use splenectomy w/ chronic ITP

50
Q

MCC of ITP

A

Idiopathic

Can also be viral or drug-induced tho

51
Q

Passive autoimmune thrombocytopenia

A

Mother w/ ITP passes abs to fetus and destroys that fuckers platelets

*****MOM HAS THROMBOCYTOPENIA

52
Q

Isoimmune thrombocytopenia

A

Mom produces abs against fetus’s platelets as a result of sensitization to proteins her platelets lack (a lot like HDN)

***MOM HAS NORMAL PLATELETS

53
Q

Enlarging hemangioma, microangiopathic hemolytic anemia, thrombocytopenia, and consumptive coagulopathy

A

Kasabach-Merritt Syndrome

-is like DIC + hemangioma

54
Q

Most potent anticoagulant protein known

A

Protein C

  • Homozygous deficiency =» Purpura fulminanas, fever, shock, intravascular thromboses
  • Heterozygous deficiency =» DVTs

Tx: Heparin, FP, warfarin

55
Q

Purpura fulminans

A

Nonthrombocytopenic purpura

-MC initial presentation of Protein C deficiency

56
Q

Noncyclic neutropenia in childhood

A

Chronic Benign Neutropenia of Childhood

-Increased incidence of sinusitis, pharyngitis, cellulitis, but otherwise healthy

Labs: Immature PMNs

-Usually resolves spontaneoussly

57
Q

Kostmann Syndrome

A

Severe congenital agranulocytosis w/ frequent life-threatening bacterial infxns; counts are usually

58
Q

Cyclic neutropenia

A

What it sounds like; pts. have fever, oral ulcers, and stomatitis during the 21 day cycle of neutropenia

Dx: Serial neutrophil counts displaying the cyclical nature of neutropenia

59
Q

Oculocutaneous albinism, blue-gray granules in PMNs, neutropenia, blond hair

A

Chediak-Higashi syndrome

  • AR disorder
  • Increased risk of severe infxn
60
Q

Short stature, immunodeficiency, fine hair, and neutropenia

A

Cartilage-hair hypoplasia syndrome

AR disorder

61
Q

Autoimmune neutropenia

A

Anti-PMN-antibodies produced in response to infxn, SLE, drugs RA

62
Q

Isoimmune neutropenia

A

Mom transfers anti-PMN-abs to the fetus; neutropenia resolves by 8 weeks of life

Pediatrics (9 decks)

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