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Peds > Heme > Flashcards

Heme Flashcards

1
Q

Peutz-Jeghers syndrome has what features? What is management?

A

Autsomal dominant unregulated tissue growth (particularly GI)

Hyperpigmented mucocutaneous macules (lips, buccal mucosa, palms/soles)
Hamartomatous polyps in GI tract –> occult bleeding or intestinal obstruction
Increased risk for GI, breast, genital tract cancers

Management: Annual anemia screening, cancer screening (eg upper/lower endoscopy)

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2
Q

Desmopressin can be used for what coagulopathy?

A

von Willebrand disease

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3
Q

Anti-D Ig can be first-line therapy for what coagulopathy?

A

ITP - only works if they are Rh(D)+ and Coombs-negative

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4
Q

Who is more likely to develop chronic ITP (lasts >1 year) rather than <3 months self-resolved? What is appropriate testing and management?

A

Those with mild initial presentation and no preceding viral trigger
Bone marrow exam and blood tests for infection and autoimmune disease; if negative, management similar to ITP

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5
Q

What is first-line therapy for ITP?

A

Children: Glucocorticoids, IVIG, or anti-D if bleeding; otherwise observe if cutaneous only (platelet cutoff of 30,000 applies to adults only)

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6
Q

What is second-line therapy for chronic ITP?

A

Rituximab
Thrombopoietin receptor agonists

Splenectomy if refractory

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7
Q

Treatments for hereditary spherocytosis

A

Folic acid supplementation
Blood transfusion
Splenectomy

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8
Q

What tests are used to confirm hereditary spherocytosis?

A

Acidified glycerol lysis (osmotic fragility)
Eosin-5-maleimide binding tests

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9
Q

Why can polycythemia of newborn cause?

A

Respiratory distress, cyanosis, apnea
Irritabiliy, jitteriness
Hypoglycemia, hypocalcemia - due to increased cellular uptake

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10
Q

Name the Hemoglobin of these diseases:
Normal
Beta-thalassemia minor
Beta-thalassemia major
Sickle cell trait
Sickle cell disease

A

Normal: Hgb A (2 alpha 2 beta)
Beta-thalassemia minor: Increased Hgb A2 (2 alpha 2 delta), decreased Hgb A
Beta-thalassemia major: Vastly increased Hgb A2, F; absent Hgb A
Sickle cell trait: Increased Hgb S, vastly decreased Hgb A
Sickle cell disease: Vastly increased Hgb S, F; absent Hgb A

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11
Q

What is a skeletal consequence of untreated beta thalassemia major?

A

Skeletal abnormalities due to extramedullary hematopoiesis

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12
Q

What is a complication of beta thalassemia major treatment?

A

Blood transfusion-dependent iron overload - use chelation therapy to avoid damage to liver, kidneys, and endocrine glands

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13
Q

What causes the destruction of cartilage and bone in hemophilic arthropathy?

A

Hemosiderin deposition –> synovial inflammation –> fibrosis and destruction
Best seen on MRI but may be visible on x-ray if severe

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14
Q

What is the mechanism of G6PD deficiency RBC death?

A

Lack of NADPH –> lack of glutathione –> oxidation –> denature/precipitate Hgb into Heinz bodies –> reduced RBC flexibility –> cell damage (bite cells) and lysis in reticuloendothelial system

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15
Q

What is the mechanism of penicillin and cephalosporin-induced immune-mediated hemolytic anemia?

A

Bind to RBC surface and act as hapten for IgG attachment –> RBC destruction by splenic macrophages –> extravascular hemolysis

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16
Q

What virus can cause aplastic crisis of RBCs in those predisposed to RBC lysis (hereditary spherocytosis, sickle cell disease, etc.)?

A

Parvovirus

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17
Q

Sickle cell trait has what most common renal issue? What are less common renal and non-renal issues?

A

Microscopic/gross hematuria from sickling in renal medulla
Hyposthenuria - impairment in concentrating ability - nocturia and polyuria

Less common:
UTI, particularly during pregnancy
Splenic infarction at high altitudes

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18
Q

Hereditary spherocytosis is more common in what population?

A

Northern European, autosomal dominant

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19
Q

What is paroxysmal nocturnal hemoglobinuria? Manifestations?

A

Acquired, complement-mediated hemolytic anemia

Hemolytic anemia
Cytopenias
Hypercoagulability

Diagnosed by CD55 and CD59 absence

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20
Q

What does dactylitis in an infant suggest?

A

Vaso-occlusive crisis in sickle cell disease
Also has erythema and warmth, may be preceded by trigger (eg dehydration)
+/- low-grade fever

Older patients should be considered for autoimmune disorders (eg psoriatic arthritis, ankylosing spondylitis)

21
Q

Sickle cell predisposes to what bacterial osteomyelitis?

A

Salmonella

S aureus is most common cause in all patients

22
Q

Hemoglobin < what is concerning for pathologic etiology in infant?

A

<9; remember that physiologic anemia of infancy can present with nadir around 11 at age 2-3 months in term infants

23
Q

How do TTP and ITP treatments differ?

A

TTP: Plasma exchange (removes anti-ADAMTS13 Ab and replaces ADAMTS13)
ITP: IVIG

24
Q

Heinz bodies of G6PD deficiency appear on what stain?

A

Special crystal violet stain (not Wright stain)

25
Q

What RBC observation on peripheral smear is due to asplenia or functional hyposplenism?

A

Howell-Jolly bodies on Wright stain - RBC nuclear remnants normally removed by spleen

26
Q

What would you observe on peripheral smear with lead poisoning?

A

Basophilic stippling - multiple, scattered, blue granules within RBC cytoplasm
Due to precipitation of ribosomal ribonucleic acid

27
Q

Infant iron deficiency treatment

A

Oral iron therapy; recheck Hgb in 4 weeks; if risen 1, then continue iron for 2-3 months after Hgb normalizes

28
Q

Is G6PD deficiency managed with splenectomy?

A

No - avoidance of hemolytic triggers

29
Q

Testicular enlargement can be seen in what heme malignancy?

A

ALL - along with SVC syndrome and pancytopenia

30
Q

As opposed to in children, where can ALL potentially be located in adolescents and adults?

A

Thymus - anterior mediastinal mass - T-ALL

31
Q

How to calculate corrected reticulocyte count?

A

retic x hematocrit/45%

32
Q

What is treatment for hyperbilirubinemia in newborn after phototherapy?

A

Exchange transfusion - unconjugated bilirubin >25 or if rapid rate of rise unresponsive to phototherapy

33
Q

Hydroxyurea side effects

A

Macrocytosis
Myelosuppression

34
Q

How is MCHC affected in:
Iron deficiency anemia
Beta thalassemia
Hereditary spherocytosis

A

Decreased in IDA, beta thalassemia
Increased in hereditary spherocytosis

35
Q

What does vitamin K do?

A

Essential for enzymatic carboxylation and activation of II, VII, IX, and X

36
Q

How do platelets appear in Bernard-Soulier syndrome?

A

Unusually large, low platelets
Prolonged bleeding time

Due to mutation in GPIb-alpha, GPIb-beta, and GPIX

37
Q

What mutation causes cyclic neutropenia or severe congenital neutropenia (as opposed to benign familial neutropenia)? What is treatment?

A

Mutations in neutrophil elastase

Treat with G-CSF

38
Q

What lab count is the definition of neutropenia? What is severe and less likely to be due to myelosuppression?

A

ANC <1500

ANC <500 is severe
ANC <200 seen in severe congenital neutropenia, along with monocyte elevation

39
Q

Pulse ox sat ~85% without further improvement suggests what?

A

Methemoglobinemia - Fe+3 –> Hgb structure change –> increased affinity for O2 at other sites –> left shift –> decreased oxygen delivery
85% is due to absorption of light at different wavelengths

Will also see >5% saturation gap between pulse ox and ABG - due to ABG only analyzing unbound arterial oxygen, showing falsely elevated O2 saturation, shown as normal PaO2

40
Q

What is co-oximetry testing?

A

Analyzes hemoglobin absorption wavelengths for Hgb, methemoglobin, and carboxyhemoglobin

41
Q

Methemoglobinemia treatments

A

Methylene blue - reduced by NADPH so that it can reduce methemoglobin
High-dose vitamin C if unavailable or contraindicated (G6PD deficiency)

42
Q

What is the side effect of hydroxyurea?

A

Macrocytic aplasia - inhibits ribonucleoside reductase, preventing creation of DTP
Myelosuppression, low reticulocyte count

43
Q

What is Mentzer index?

A

MCV/RBC ratio
<13: suggests thalassemia

44
Q

Increased erythrocyte adenosine deaminase activity

A

Diamond-Blackfan anemia

45
Q

Diamond-Blackfan anemia treatment

A

Steroids if >=1 yo, RBC transfusions if <1 yo or steroid-resistant; consider BMT

46
Q

Fanconi anemia treatment

A

Allogeneic hematopoietic cell transplantation
Androgen therapy (e.g. oxymetholone), growth factors (e.g. G-CSF, GM-CSF), blood transfusion

47
Q

Diamond-Blackfan vs Fanconi anemia - inheritance

A

D-B: Autosomal dominant
Fanconi: Autosomal recessive or X-linked

48
Q

RBM8A gene

A

Thombocytopenia absent radius syndrome - autosomal recessive

Hypomegakaryocytic thrombocytopenia and bilateral radial bone aplasia in presence of thumbs

49
Q

Kasabach-Merritt syndrome

A

Life-threatening coagulopathy of infancy like DIC - attributed to aggressive benign vascular tumors

Can cause reactive hemarthrosis, congestive heart failure, GI bleeding

Would see enlarging, firm, purpuric cutaneous or soft tissue lesions; do not biopsy due to bleeding risk

Surgical resection is definitive

Peds (25 decks)

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