Heme Flashcards

1
Q

Microcytic anemias

A

(<80) → iron deficiency,thalassemia, lead tox, neoplastic

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2
Q

Normocytic anemias

A

(80-100) → acute blood loss, anemia of chronic dz, hemolyic, renal dz

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3
Q

Macrocytic anemias

A

(>100) → hemolytic, alcohol, liver dz, aplastic anemia, hypothyroidism, drug effect, myseldysplasia

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4
Q

Megaloblastic anemias

A

(>110) → folate deficiency, B12 deficiency

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5
Q

When should you screen for lead poisoning

A

Screen at 10-14 mo and 2 yo

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6
Q

Tx for lead poisoning

A

Enviro control, education

10-19 → deu
20-45→ enviro eval +
45-69 → med and enviro intervention including chelation (EDTA or DMSA)
>70 → medical emergency Dimercaprol followed b EDTA x 5 days

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7
Q

Presentation of lead poisoning

A

Acute (>60) → HA, abd pain, loss of appetite, constipation, acute encephalopathy

Chronic → cognitive impairment, ADHD

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8
Q

Hemophilia presentation

A

Easy bruising, intramuscular hematomas, hemarthroses → joint destruction, spontaneous hemorrhaging

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9
Q

Hemophilia A

A

factor VIII deficiency

MC than B and is more likely to be severe

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10
Q

Hemophilia B

A

factor IX deficiency

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11
Q

Hemophilia tx

A

Early diagnosis, prevent trauma

Prompt mgmt of bleeding, recombinant factors, cryoprecipitate

AVOID anticoag, aspirin and NSAIDs

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12
Q

ALL presentation

A

Fatigue, anorexia, lethargy, pallor, bone pain, fever, bleeding, bruising, petechiae, lymphadenopathy, hepatosplenomegaly, bone tenderness,testicular swelling, septicemia

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13
Q

What leukemia is mc

A

ALL 5x mc than AML

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14
Q

Leukemia tx

A

Remission induction: 1 wk anthracycline and cytosine arabinoside

Postremission therapy: several more courses of high dose cytrabine chemo, allogenic stem cell transplant or autologous stem cell transplant

Infection prevention→ isolation and abx

Platelet transfusion for bleeding

RBC trans for anemia

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15
Q

Juvenile Chronic Myelogenous Leukemia (JCML) presentation

A

<2 yo

Skin lesions (eczema, xanthoma, café au lait spots), lymphadenopathy, splenomegaly

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16
Q

Juvenile Chronic Myelogenous Leukemia (JCML) tx

A

Complete remission w/ stem cell transplant

Majority relapse with overallsurvival of 25%

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17
Q

Lymphoma presentation

A

MC childhood cancer in 15-19 yo

Fever, night sweats, weight loss, loss of appetite, cough, dysphagia, dyspnea, lymphadenopathy (lower cervical and supraclavicular), hepatosplenomegaly, mediastinal mass

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18
Q

reed-sternberg cells (owl eyes)

A

Hodgkin lymphoma

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19
Q

Lymphoma tx

A

Radiation for stage I or II dz

Chemo for stage III or IV

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20
Q

Neutropenia w/u

A

ANC < 1500

Mild: 1000-1500

Moderate: 500-1000

Severe: <500

Agranulocytosis: <200

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21
Q

Neutropenia presentation

A

suspectibility ot bacterial infection

Stomatitis, gingivitis, recurrent OM, cellulitis, pneumonia and septicemia

Skin and oral cavity mc affected

22
Q

Juvenile pernicious anemia, red beefy tongue

A

vit B12 def anemia

23
Q

tx for vit B12 def anemia

A

Vit B12 IM monthly

Oral therapy is CI

24
Q

tx for iron def anemia

A

Ferrous sulfate 3-6 mg/kg/day x 8 wks after a nrml Jgb level is obtained

25
Q

tx for folate def anemia

A

Parenteral folic acid (CI if co-existing vit B12 deficiency anemia)

Eat more green veggies, fruits

26
Q

Appears after 6 mo (when HbF↓)

Anemia, vaso-occlusive (leg ulcers, stroke,priapism, pain crises), hand foot syndrome (swollen), splenomegaly, infection, cardiac enlargement, short stature, delayed puberty, gallstones/jaundice

A

Sickle Cell Disease

27
Q

Sickle Cell Disease tx

A

Infection prevention → pneumococcal vaccine, meningococcus, pphx Pcn by 4 m until 5 yo

Hydration and analgesics for painful crisis

Priapism → exchange transmusion, hydroxyurea

28
Q

Hemolytic Disease of the Newborn presentation

A

Hemolytic anemia

Fetal hydrops→ large placenta, ↑ unconjugated hyperbilirubinemia, abd distension,scalp or skin edema, purpura, cyanosis

29
Q

Hemolytic Disease of the Newborn etiology

A

Maternal blood comes in contact w/ fetal blood cells → maternal Ab produced against Rh Ag → subsequent preg w/ Rh + → hemolysis

30
Q

Thalassemia tx

A

Monthly transfusion of packed RBC to maintain Hgb >10

Splenectomy if requiring >240 mL/k of pRBC/yr

31
Q

Aplastic Anemia

A

pancytopenia (RBC WBC and platelets)

Fatigue, infections, bleeding

32
Q

Aplastic Anemia tx

A

Platelet and RBC transfusion

Immunosupp drugs → antilymphocyte globulin, antithymocyte globulin, cyclosporine

Growth factors→ EPO, G-CDF, GM-CSF

Stem cell trasnplant is definitive cure but required chemo and/or radiation

33
Q

Thrombotic Thrombocytopenic Purpura (TTP) presentation

A

Fever, microangiopathic hemolytic anemia, thrombocytopenia, abnrml renal fn, neurologic signs

34
Q

TTP tx

A

Plasmapheresis, corticosteroids, splenectomy

35
Q

HUS presentation

A

Hemolytic anemia, thrombocytopenia, acute renal failure

Hx of bloody diarrhea

36
Q

HUS tx

A

Fluid mgmt, dialysis, plasmapheresis

37
Q

ITP presentation

A

Abrupt onset of petechial, purpura and epistaxis

Usually 1-4 wks after viral infection

38
Q

ITP tx

A

If platelet <20 admit

IVIG or anti-Rho Ab

Splenectomy

Platelet transfusion

39
Q

DIC presentation

A

Bleeding, petechia, ecchymoses, hemolysis

40
Q

DIC tx

A

Treat underlying cause

replace plateleys, cryoprecipitate, FFP

Heparin prevents consumption of coag factors

41
Q

Von Wilebrand Disease presentation

A

Easy bruising, heavy or prolonged menstruation, freq or prolonged epistaxis, prolonged bleeding after inj

42
Q

Von Wilebrand Disease w/u

A

aPTT nrml or prolonged

Prolonged bleeding time

Nrml PT

Abnrml factor VIII , platelet count and aggregation

43
Q

Von Wilebrand Disease tx

A

Usually no therapy necessary

Avoid unnecessary trauma

DDAVP and favtor VIII for surgery if needed

Cryoprecipitate recommended only in life threatening emergencies d/t risk of HIV and hepatitis infection

44
Q

What is MC posterior fossa tumor of childhood

A

Cerebellar Astrocytoma

45
Q

Prognosis of Cerebellar Astrocytoma

A

Good prognosis

5 yr survival >90%

46
Q

Medulloblastoma presentation

A

Intracranial HRN, ataxiam sx evolving in few weeks

Papilledema is absent

47
Q

Craniophryngioma presentation

A

Short stature or other endocrine associated problems are common initial signs

May compress optic nerve or obstruct CSF flow

48
Q

Neuroblastoma presentation

A

Adrenals, retroperitoneal and abd are MC

49
Q

Neuroblastoma tx

A

Surgical resection followed by radio + chemotherapy

50
Q

Meduloblastoma tx

A

Surgical resection followed by irradiation

5 yr survival is >80%