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1. PPS Board Prep_Mar > HEMA > Flashcards

HEMA Flashcards

1
Q

Diagnostic criteria for DIAMOND-BLACKFAN SYNDROME (4)
Major criteria (2)

A

PRAM
1. Age <1yo
2. Macrocytic anemia
3. Reticulocytopenia
4. Paucity of BM erythroid precursors

Major:
1. Pathogenic mutations
2. Positive family hx

Minor:
1. Elev eADA
2. Congenital anomalies of DBA
3. Elev HbF
4. No other evidence of inherited BM failure syndromes

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1
Q

DBA vs TEC

A

DBA / TEC
> Onset: early / late (after viral infx)
> Lab: Macrocytic / normal
> eADA: elev / normal
> PE: congeniial abn / normal

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2
Q

Mainstay therapy of DBA

A

Corticosteroids (Prednisone/Prednisolone 2mkday)
> Inc RBC precursors in 1-3wks)

delayed until after 1yo because steroids impair linear growth and physical and neurocognitive development.

Chronic transfusion therapy.

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3
Q

Most common ACQUIRED RED CELL APLASIA

A

Transient erythroblastopenia of childhood

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4
Q

TRUE or FALSE.
Corticosteroid therapy is the mainstay treatment for Transient Erythroblastopenia of childhood.

A

FALSE.
> Corticosteroids are of no value in TEC.
> TEC resolves spontaneously after 1-2mos

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5
Q

Best documented viral cause of red cell aplasia in px w chronic hemolysis, immunocompromised and fetuses in utero

A

Parvovirus B19
> erythema infectiosum (fifth dse)
> attach to P antigen in RBC
> infective and cytotoxic to marrow cells
> cause aplastic crisis in px w hemolytic dse - severe anemia

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6
Q

Mechanisms contributing to ACD (3)

A
  1. Decreased RBC life span
  2. Impaired erythropoiesis
  3. Decreased iron uptake by RES
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7
Q

Most common form of Congenital Dyserythropoietic Anemia

A

Type II.
> Autosomal recessive
> AKA Hereditary Eryhtroblastic multinuclearity with positive acidified serum test (HEMPAS)
> May benefit from splenectomy
> normocytic
> Presence of pseudo-gaucher cells

Type I
> AR
> macrocytic
> Swiss Cheese hemochromatic pattern on electron microscopy
> Tx is supportive

Type 3
> AD
> rare
> no iron overload

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8
Q

Starting dose of iron therapy for anemia of prematurity

A

1-2mkday elemental Fe
> starting 1 month until 1yo

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9
Q

Diagnostic test to differentiate IDA from ACD

A

Soluable transferring Receptor (STfR)
> elevated in IDA

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10
Q

What level of hgb manifests as irritability, lethargy, anorexia and systolic flow murmurs?

A. <5g/dL
B. <6
C. <7
D. <9

A

A. <5g/dL

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11
Q

Most accurate diagnostic test for IDA

A

Bone Marrow iron staining
> invasive

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12
Q

IDA: inc or dec

  1. Serum ferritin
  2. Serum transferrin
  3. TIBC
  4. FEP
  5. RDW
  6. MCV
  7. MCH
  8. STfR
A

PATHOPHYSIOLOGY:
> Tissue iron decrease
> Serum iron decrease = DEC serum ferritin (iron storage protein)
> INC serum transferrin (Binding protein) and INC TIBC, and DEC serurm transferrin saturations
> not enough iron to complex w protoporhyrin to form heme therefore
> INC FEP

Resulting in elliptocytes/cigar sshaped RBC (INC RDW), hypochromic (dec MCH) and microcytic (DEC MCV) and DEC RBC count

MCV is a LATE indicator for iron def

INC soluable transferring receptor (STfR) in IDA

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13
Q

True or False. MCV is a late indicator for Iron deficiency

A

TRUE
> MCV is a reliable but late indicator for IDA

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14
Q

Which indicator for IDA is affected by diurnal variation of serum iron?

A. TIBC
B. Transferrin saturation
C. MCV
D. Serum ferritin
E. Reticulocyte hgb conc.

A

B. Transferrin saturation
> limited by diurnal variation of serum iron and clinical conditions including inflammation, aging and nutrition

A. TIBC
C. MCV - late indicator for ID
D. Serum ferritin - MOST USEFUL. Also an acute phase reactant.
E. Reticulocyte hgb conc - sensitive indicator and not affected by inflammation

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15
Q

After how long would you expect bone marrow response and erythroid hyperplasia?

A. 12-24hrs
B. 36-48hrs
C. 48-72hrs
D. 4-30days

A

B. 36-48hrs

A. 12-24hrs: subjective improvement, replacement of intracellular iron stores, dec irritability, inc appetite and inc serum iron
C. 48-72hrs: Reticulocytosis, peak 5-7days
D. 4-30days: Inc hgb, MCV and ferritin

1-3mos: Repletion of stores

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16
Q

Dose of iron for IDA

A

3-6mg/kg of elemental iron daily in 1 or 2 doses

Iron therapy can enhance malarial and G Neg bacterial infections. Iron overdose asstd w Yersinia infx

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17
Q

Expected hgb elevation daily once iron therapy started

A

After month: Inc by 1-2g/dL

Daily: 0.1-0.4g/dL/day

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18
Q

Duration of antibiotic prophylaxis post-splenectomy for hereditary spherocytosis?

A

Prophylactic oral Penicillin until 5yo or 2yrs after splenectomy

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19
Q

What blood disorder is protective of Falciparum malaria?

A

G6PD
> Evolutionary advantage of resistance of falciparum malaria in heterozygous females

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19
Q

Heinz bodies is appreciated on PBS for what blood disorder?

A

G6PD Deficiency
> “bite cells” also present

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19
Q

Vitamin K dependent coagulation factors. (4)

A

CF II, VII, IX, X

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19
Q

CF deficiency inheritance
1. Autosomal dominant (2)
2. Sex-linked recessive (2)

A
  1. Autosomal dominant: CF 1 and 12 deficiency
  2. Sex-linked recessive: CF 8 and 9 deficiency
19
Q

MOST COMMON inherited Bleeding disorder

A

Von Willebrand disease

20
Q

Treatment of vWF disease

A
  1. DDAVP (desmopresin)
  2. Plasma derived vWF
  3. E-aminocaproic acid - antifibrinolytic agent
21
Q

IEM associated w megaloblastic anemia

A

Orotic aciduria
> Dev delay
> megaloblastic anemia
> growth retardation
> inc urinary excretion of orotic acid
> most common metabolic error in the de novo synthesis of pyrimidines

22
Q

Which anemia has been identified as the precancer syndrome?

A. Pearson Syndrome
B. Diamond Blackfan Anemia
C. Iron deficiency anemia
D. Vit B12 deficiency

A

B. Diamond Blackfan Anemia
> higher risk of developing myelodysplastic syndrome, AML, colon CA, osteogenic sarcoma and female genital cancers

23
Q

Most severe form of:
Alpha thalassemia

Beta thalassemia

A

> Alpha thalassemia: Hydrops fetalis
Beta thalassemia: cooley anemia

24
Q

Most common leukemia

A
  1. ALL (70%)
  2. AML
  3. CML
  4. CLL
25
Q

1st line treatment for CML

A

Tyrosine kinase inhibitor: Imatinib

26
Q

This chronic leukemia LACKS the Philadelphia chromosome

A

Juvenile Myelomonocytic Leukemia
> Rare
> occurs in less than 2yo
> Asstd w Noonan syndrome and NF1

Tx: HSCT

27
Q

Top 3 childhood cancers

A
  1. Leukemia
  2. CNS Tumors
  3. Lymphomas
28
Q

Hallmark/classic histologic LN findings in Hodgkin Lymphoma

A

Reed Sternberg cells
> owl’s eye within a lymphocyte blanket

29
Q

Treatment of Hodgkin Lymphoma

A

4-6 cycles of ABVE
Adriamcyin (Doxorubicin)
Bleomycin
Vincristine
Etoposide

30
Q

Classic Xray finding on Osteogenic Sarcoma

A

Sunburst pattern
> Periosteal lifting and Codmans triangle
> Soft tissue mass + calcification

31
Q

Class Xray finding for Ewing Sarcoma

A

> Onion-skinning
Moth-eaten lytic lesions
“Yung moth may eWINGs 🪽”

32
Q

Most common solid tumor of INFANCY

A

Neuroblastoma
> Racoon’s eyes (periorbital eccymoses)
> Inc VMA in urine (urinary catecholamines)
> Fever+wt loss

33
Q

BMA findings in Neuroblastoma

A

Homer Wright pseudorosette

34
Q

TRUE or FALSE. Osteosarcoma is both chemo and radiosensitive

A

FALSE.
Between Osteosarcoma and Ewing, Ewing is radiosensitive.

35
Q

6 oncologic emergencies

A
  1. Hyperleukocytosis (100,000) - risk for leukostasis
  2. Tumor Lysis Syndrome - hyper PUK!
    > Elev: Ph, Uric acid, K
  3. SVC syndrome
  4. Spinal cord compression
  5. Cardiac tamponade
  6. Neutropenic enterocolitis/typhilitis
36
Q

Which of the ff is a medical emergency in children w Sickle Cell disease?

A. Hemolysis
B. Bleeding
C. Fever
D. Thrombosis

A

C. Fever
> As early as 6mos of age, infants w sickle cell anemia develop abnormal immune function because of splenic dysfunction
> IV antibx: 3rd gen ceph (Ceftriaxone)
> Risk of infx from: encapusulated bacteria: S. pneumoniae, H. influenza B, N. meningitidis

37
Q

Life-threatening complication of Sickle cell disease:

A. Acute chest syndrome
B. Acute splenic sequestration
C. Hand foot syndrome
D. A and B

Tx?

A

D. A and B

Acute chest syndrome
> new radiodensity on CXR plus 2 of the ff:
1. fever
2. respi distress
3. hypoxia
4. cough
5. chest pain

Acute splenic sequestration
> may occur as early as 5wk old, most often occurs bet 6mos-2yo
> Rapid spleen enlargement causing left-sided abdominal pain
> Hb decline at least 2g/dL from baseline
> Hypovolemia, anemia, (<33g/dL), dec WBC and plt
> Tx: correct hypovolemia - Isotonic fluid or blood transfusion
> BT aborts blood trapping in the spleen and allows release of the px RBC that have become sequestered in the spleen
> BT 5ml/kg or target hgb 8g/dl

> Prophylactic splenectomy after acute episode
Complication is hyperviscosity

38
Q

Most often the first manifestation of pain in Sickle Cell disease

A. Acute chest syndrome
B. Dactylitis
C. Aplastic crisis

A

B. Dactylitis

> AKA Hand foot syndrome
50% beyond 2yo
symmetrical or unilateral swellingg of the hands or feet

39
Q

Cardinal clin fx of sickle cell disease

A

Acute vasoocclusive pain

40
Q

Classic Fanconi Anemia phenotype triad

A
  1. Bone marrow failure
  2. Congenital anomalies
    > MC skeletal abn: absent radii/thumb- hypoplastic, supernumerary, bifid or absent
  3. Chromosomal fragility
41
Q

Hallmark of hemophilic bleeding is ___.

A

Hemarthroses

42
Q

Pentad of Trombotic Thrombocytopenic Purpura

A

MFAT
1. fever
2. microangiopathic hemolytic anemia
3. thrombocytpenia
4. Abnormal renal and CNS fx

> Tx is plassmapheresis
caused by auto-ab mediated deficiency of ADAMTS133

43
Q

What syndrome?
> Giant hemangioma w localized intravascular coagulation
> hypofibrinogenemia
> thrombocytopenia

A

Kassabach-Merritt Syndrome

44
Q

What syndrome?
1. thrombocytopenia
2. eczema
3. recurrent infections as a consequence of immune deficiency

A

Wiskott Aldrich Syndrome

45
Q

The ff are more commonly observed in:
A. ALL
B. AML
C. Both

subcutaneous nodules
gingival infiltration
DIC

A

B. AML
Presents w s/sx uncommon in ALL:
> Subcutaneous nodules or blueberry muffin lesions (infants)
> gingival infiltration
> DIC
> discrete massess - chloroma or granulocytic sarcomas

46
Q

MC malignant primary renal tumor

A

Wilms Tumor
> 2nd most malignant abdominal tumor (1st neuroblastoma)

47
Q

MC malignant abdominal tumor

A

Neuroblastoma
> embryonal cancer of the PNS

48
Q

Indicators of accelerated RBC destruction (3)

A
  • reticulocytosis
    • indirect hyperbilirubinemia
    • increased serum lactate dehydrogenase
49
Q

MC benign tumor of infancy

A

Hemagioma

50
Q
A

1. PPS Board Prep_Mar (16 decks)

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