APHON CHemOtHerAPy CertifiCAtiON exAm –
LAtest editiON witH A+ GrAded QuestiONs &
ANswers.
Hereditary spherocytosis - Answer: >>> Inherited, non-immune
hemolytic anemia that involves cell membrane alterations that causes fragile
RBC to be trapped in spleen - shortens RBC life span
In hereditary spherocytosis RBC shaped like - Answer: >>> Spheres,
smaller in diameter, more rigid, shorter life psna (10-90 days)
HS is mostly autosomal - Answer: >>> Dominant
Classic sign of HS - Answer: >>> red blood cell destruction
How is spleenomegaly with HS? - Answer: >>> Mild
Lab in newborns that almost always means HS - Answer: >>> High mean
corpuscular hemoglobin concentration
One odd lab for HS - Answer: >>> Positive osmotic fragility test
Treatment before age of 5 for HS and what's rarely needed - Answer:
>>> Folic acid / transfusions
Page 1 of 176
,APHON CHemOtHerAPy CertifiCAtiON exAm –
LAtest editiON witH A+ GrAded QuestiONs &
ANswers.
Aplastic anemia - / Cytopenia - / Pancytopenia - - Answer: >>> Complete
arrest of bone marrow / one cell line fails to produce (Diamond Blackfan) / More
than one cell line (Fanconi and dyskeratosis congenita)
Aplastic anemia - - Answer: >>> Peripheral blood pancytopenia and
hypocellular bone marrow
Acquired AA caused by? - Answer: >>> Idiopathic or
chemicals/meds/radiation/parvo
Labs for AA (3) - Answer: >>> CBC shows decrease in 1/more cell lines,
noraml RBC morphology, retic count low
AA classifications - with ANC and Platelets
A) Moderate
B) Severe
C) Very severe - Answer: >>> A) < 1200 and Plts < 100,000
B) ANC < 500 and Plts < 20,000
C) ANC < 200 and Plts < 20,000
Treatment for severe AA vs when can't HLA match - Answer: >>> HLA
matched sib transplant / ATG and CYA, corticosteroids, GCSF
Page 2 of 176
,APHON CHemOtHerAPy CertifiCAtiON exAm –
LAtest editiON witH A+ GrAded QuestiONs &
ANswers.
Inherited AA most common causes - Answer: >>> Fanconi anemia - most
common, dyskeratosis congenita, Schwachman-Diamond sydnrome
FA is inherited y - Answer: >>> Autosomal recessive or X-linked disease -
both parents must carry gene
Symptoms of FA (13) - Answer: >>> Skin pigment change, short stature,
UA anomalies, small testicle, scoliosis, microcephaly, eye anomalies, ear
anomalies, broad nose, Kidney malformation, GI/cardiopulmonary
malforatmion, learning disability, FTT
Labs with FA (4) - Answer: >>> Anemia, thrombocytopenia, neutropenia,
chromosome breakage analysis through DEB or MMC
Treatment for FA - Answer: >>> HSCT
Supportive therapy for FA (3) - Answer: >>> Androgen therapy, GCSF,
steroids
Pts with FA more sensitive to (2) - Answer: >>> Carcinogens and radiation
(x-rays and CT scans)
Page 3 of 176
, APHON CHemOtHerAPy CertifiCAtiON exAm –
LAtest editiON witH A+ GrAded QuestiONs &
ANswers.
Dyskeratosis congenita - - Answer: >>> Inherited associated with
progressive bone marrow failure characterized by triad of dermatologic
symptoms
Triad of dermatologic s/s associated with dyskeratosis congenita - Answer:
>>> Skin hyperpigmentation, nail dystrophy, leukoplakia
Cellular level of DC (3) - Answer: >>> Telomerase dysfunction, protein-
syntehsis dysfunction, ribosome deficiency
Other S/s of DC (6) - Answer: >>> Pulmonary fibrosis, eye abnormalities,
abnormal dentition, short stature, hypospadias
First sign of bone marrow failure in DC - Answer: >>> Thrombocytopenia
- progression to complete bone marrow failure always occurs
How to determine difference between DC and FA - Answer:
>>> Chromosome breakage analysis in FA and short telomeres in DC
Treatment for DC and what should they not receive - Answer: >>> HSCT
and busulfan
Page 4 of 176
LAtest editiON witH A+ GrAded QuestiONs &
ANswers.
Hereditary spherocytosis - Answer: >>> Inherited, non-immune
hemolytic anemia that involves cell membrane alterations that causes fragile
RBC to be trapped in spleen - shortens RBC life span
In hereditary spherocytosis RBC shaped like - Answer: >>> Spheres,
smaller in diameter, more rigid, shorter life psna (10-90 days)
HS is mostly autosomal - Answer: >>> Dominant
Classic sign of HS - Answer: >>> red blood cell destruction
How is spleenomegaly with HS? - Answer: >>> Mild
Lab in newborns that almost always means HS - Answer: >>> High mean
corpuscular hemoglobin concentration
One odd lab for HS - Answer: >>> Positive osmotic fragility test
Treatment before age of 5 for HS and what's rarely needed - Answer:
>>> Folic acid / transfusions
Page 1 of 176
,APHON CHemOtHerAPy CertifiCAtiON exAm –
LAtest editiON witH A+ GrAded QuestiONs &
ANswers.
Aplastic anemia - / Cytopenia - / Pancytopenia - - Answer: >>> Complete
arrest of bone marrow / one cell line fails to produce (Diamond Blackfan) / More
than one cell line (Fanconi and dyskeratosis congenita)
Aplastic anemia - - Answer: >>> Peripheral blood pancytopenia and
hypocellular bone marrow
Acquired AA caused by? - Answer: >>> Idiopathic or
chemicals/meds/radiation/parvo
Labs for AA (3) - Answer: >>> CBC shows decrease in 1/more cell lines,
noraml RBC morphology, retic count low
AA classifications - with ANC and Platelets
A) Moderate
B) Severe
C) Very severe - Answer: >>> A) < 1200 and Plts < 100,000
B) ANC < 500 and Plts < 20,000
C) ANC < 200 and Plts < 20,000
Treatment for severe AA vs when can't HLA match - Answer: >>> HLA
matched sib transplant / ATG and CYA, corticosteroids, GCSF
Page 2 of 176
,APHON CHemOtHerAPy CertifiCAtiON exAm –
LAtest editiON witH A+ GrAded QuestiONs &
ANswers.
Inherited AA most common causes - Answer: >>> Fanconi anemia - most
common, dyskeratosis congenita, Schwachman-Diamond sydnrome
FA is inherited y - Answer: >>> Autosomal recessive or X-linked disease -
both parents must carry gene
Symptoms of FA (13) - Answer: >>> Skin pigment change, short stature,
UA anomalies, small testicle, scoliosis, microcephaly, eye anomalies, ear
anomalies, broad nose, Kidney malformation, GI/cardiopulmonary
malforatmion, learning disability, FTT
Labs with FA (4) - Answer: >>> Anemia, thrombocytopenia, neutropenia,
chromosome breakage analysis through DEB or MMC
Treatment for FA - Answer: >>> HSCT
Supportive therapy for FA (3) - Answer: >>> Androgen therapy, GCSF,
steroids
Pts with FA more sensitive to (2) - Answer: >>> Carcinogens and radiation
(x-rays and CT scans)
Page 3 of 176
, APHON CHemOtHerAPy CertifiCAtiON exAm –
LAtest editiON witH A+ GrAded QuestiONs &
ANswers.
Dyskeratosis congenita - - Answer: >>> Inherited associated with
progressive bone marrow failure characterized by triad of dermatologic
symptoms
Triad of dermatologic s/s associated with dyskeratosis congenita - Answer:
>>> Skin hyperpigmentation, nail dystrophy, leukoplakia
Cellular level of DC (3) - Answer: >>> Telomerase dysfunction, protein-
syntehsis dysfunction, ribosome deficiency
Other S/s of DC (6) - Answer: >>> Pulmonary fibrosis, eye abnormalities,
abnormal dentition, short stature, hypospadias
First sign of bone marrow failure in DC - Answer: >>> Thrombocytopenia
- progression to complete bone marrow failure always occurs
How to determine difference between DC and FA - Answer:
>>> Chromosome breakage analysis in FA and short telomeres in DC
Treatment for DC and what should they not receive - Answer: >>> HSCT
and busulfan
Page 4 of 176
