Newman High Yield

Question 1

Change in oxygen dissociation curve in anemia

Answer 1

shifts to right

affinity of Hb for O2 reduced in tissues needing to be oxygenated

2,3-DPG increased within RBCs

Question 2

EPO in anemia

Answer 2

levels go up, RBC production in BM increases

Question 3

MCV and peripheral smear

Answer 3

Microcytic - low MCV
Normocytic - MCV normal
Macrocytic - high MCV

Smear:  
Changes in RBC morphology:
Anisocytosis - different sizes
Poikilocytosis - different shapes
Fragments - hemolysis results in lots of hematologic trash in blood

Question 4

Flow murmur

Answer 4

with anemia, cardiac output increases

get systolic ejection murmur

Question 5

Intravascular hemolysis

Answer 5

Causes:
mechanical injury
complement fixation - autoimmune
Intracellular parasites
Toxins

Features:
anemia
*hemoglobinuria
*hemoglobinemia
*hemosiderinuria
jaundice

Question 6

Extravascular hemolysis

Answer 6

macrophages in liver and spleen eat RBCs
–> abnl abc membrane/deformilities of RBCs

Features:
*splenomegaly
anemia
jaundice

Question 7

Reticulocyte count in anemia

Answer 7

establishes if BM working

Low or normal:

• inadequate BM response to anemia
• not enough of something to make RBCs
• relative BM failure
• Ineffective erythropoiesis

Increased:

• normal BM response to anemia
• ongoing RBC destruction -hemolysis
• Sequestration
• Loss - bleeding

Question 8

Iron Deficiency Anemia

Answer 8

chronic occult blood loss or diet deficiency
excessive cow milk
inflammatory bowel dz
Meckel diverticulum
parasites

Microcytic, hypochromic
Low reticulocyte count

target cells

Fe and ferritin levels, iron saturation low
Transferrin elevated

Question 9

Lead toxicity anemia

Answer 9

microcytic, hypochromic
higher RDW - 17 or 18

low reticulocyte count
basophilic stippling

may have concurrent Fe deficiency

Question 10

Thalassemias

Answer 10

Microcytic anemia

Mediterranean
Autosomal co-dominant disorders

Question 11

Isoimmunization

Answer 11

positive direct coombs test
elevated indirect bilirubin
normocytic anemia
elevated reticulocyte count

ABO incompatibility
Rh incompatibility

Question 12

Congenital hemolytic anemia

Answer 12

hemolysis
low enzyme activity
Smear: poikilocytosis, reticulocytosis, Heinz bodies, bite cells (G6PD def), spur cells (pyruvate kinase deficiency)

Question 13

Congenital infection anemia

Answer 13

normocytic anemia
Low reticulocyte count

Parvovirus B19 - anaplastic anemia
HIV, syphilis, rubella, sepsis

Question 14

Diamond Blackfan syndrome

Answer 14

macrocytic anemia with low reticulocyte count

Congenital pure red cell aplasia

Question 15

Fanconi anemia

Answer 15

microcytic anemia and reticulocytopenia, thrombocytopenia, or leukopenia

pancytopenia - BM failure syndrome
GU and skeletal abnormalities
increased Chr fragility

Question 16

*Mentzer index

Answer 16

MCV/RBC

less than 13 - thalassemia
>13 - iron deficiency

Question 17

G6PD deficiency

Answer 17

episodic hemolysis
fava beans trigger
x linked recessive

Question 18

Hereditary spherocytosis

Answer 18

AD in 75%

Heinz bodies and bite cells

Question 19

Sickle cell disease

Answer 19

AR

Holly-Jolly bodies

Question 20

Pernicious anemia

Answer 20

IF in parietal cells attacked by Ab

Question 21

*Calculate ANC

Answer 21

((%neutrophils + % bands) x (WBC))/100

Severe ANC less than 500/uL - risk of serious infection

Question 22

Cyclic neutropenia

Answer 22

Severe neutropenia less than 200 for 3-7 days every 3 weeks (15 to 35 days)

Cyclic fever, oral ulcers, gingivitis, periodontal dz, recurrent bacterial infection

Stem cell regulatory defect

Question 23

Shwachman-Diamond syndrome

Answer 23

triad: neutropenia, exocrine pancreas insufficiency, skeletal abnormalities

AR

increased risk for myelodysplastic syndrome or leukemia

Question 24

Leukocyte adhesion deficiency

Answer 24

Delayed separation of umbilical cord
recurrent and severe bacterial fungal infection without pus accumulation, poor would healing, periodontal dz

neutrophils diminished adhesion to surfaces, cannot migrate out of BV

AR

Prognosis depends on severity of deficiency

Question 25

Chronic granulomatous disease

Answer 25

recurrent purulent infections with fungal or bacterial catalase positive organisms - infancy - chronic inflammatory granulomas Defect in oxidative metabolism - absent superoxide generation nitroblue tetrazolium test X linked recessive, rarely AR Good prognosis with aggressive management of infection

Question 26

Infection associated neutropenia

Answer 26

Viruses - neutropenia within first 2-3 days of illness, last up to 1 week Decreased marrow production Depleted marrow reserves Increased margination with decreased circulating neutrophils Ab formation with increased peripheral destruction Caused by: HIV, parvovirus B19, EBV, CMV, influenza A and B, RSV...

Question 27

Drug induced neutropenia

Answer 27

cytotoxic agents for treatment of malignancies routine meds - abx, antiinflammatory, psychotropic drugs

Question 28

Leukocytosis

Answer 28

usually Reactive process to infection increased WBC, increased band cells can be d/t chronic inflammation

Question 29

Eosinophilia

Answer 29

hypersensitivity reactions, parasitic infections congenital - AD, rare Acquired: acute leukemia, MDS; 2ndary: infection, drugs, allergic disorders, autoimmune, malignancy, endocrinopathies

Question 30

Hodgkin Lymphoma

Answer 30

Red-sternberg cells - large cell with multiple or multilobulated nuclei Hematogenous spread --> liver, spleen, bone, BM, brain B symptoms: unexplained fever, wt loss >10% total body wt over 6 mo, drenching night sweats dx: persistent, unexplained LAD unassociated with inflammatory or infectious process - CXR to r/o mediastinal mass before LN bx favorable prognostic factors, early stage dz - >95% survive at 5 yrs

Question 31

Non-Hodgkin lymphoma

Answer 31

60% lymphomas in children and adolescents Predisposing conditions: SCID Wiscott-Aldrich syndrome - X linked recessive -Triad: Recurrent sinopulmonary infections, eczema, bleeding (2ndary thrombocytopenia)

Question 32

Burkett lymphoma

Answer 32

NHL abdominal (sporadic) Head and neck dz (endemic) with involvement of bone marrow or CNS

Question 33

Pathogenesis of leukemia

Answer 33

malignant transformation and clonal expansion of hematopoietic stem cells at early stage differentiation - unable to mature

Question 34

Acute leukemia

Answer 34

97% of all childhood leukemias CBC: initially high WBC --> marrow failure resulting in decreased WBC Blasts in marrow

Question 35

ALL

Answer 35

peak: 4 yr White > blacks Males > females Genetics: downs, bloom syndrome, fanconi anemia, ataxia telangiectasia, Shwachman syndrome, neurofibromatosis, twins, siblings increased risk Ionizing radiation EBV and L3 ALL Immunodeficiency - Wiskott Aldrich, congenital hypogammaglobinemia, ataxia telangiectasia

Question 36

AML

Answer 36

Increased in adolescence Genetics: downs, bloom syndrome, fanconi anemia, ataxia telangiectasia, Kostmann syndrome, NF-1, Diamond-blackfan syndrome, li-fraumeni syndrome non inherited: aplastic anemia, MDS, PNH Ionizing radiation, benzene, epipodophylotoxins, alkylating agents (nitrogen mustard, melphalan, cyclophosphamide)

Question 37

Quality of lymph nodes

Answer 37

Tender, warm, red, fluctuant - lymphadenitis Soft, mobile, smooth - benign Firm and rubbery - possible lymphoma Fixed and matted - possible malignancy or TB Generalized - 2 or more - EBV, mono not growing well - HIV

Question 38

Cat Scratch disease - eye involvement

Answer 38

parinaud's syndrome: conjunctivitis, pre auricular adenines, conjunctival granuloma neuroretinitis

Question 39

Painless testicular mass

Answer 39

w/out hx of trauma/injury get a CXR to check for likely malignancy

Question 40

Teratoma

Answer 40

sacrococcygeal most common Benign, well-differentiated cystic lesions - mature teratomas Lesions of inderterminant potential - immature teratoma Unequivocally malignant teratomas - mixed with another germ cell tumor component

Question 41

Neuroblastoma

Answer 41

tumors of sympathetic ganglia and adrenal medulla -neural crest cell origin median dx age - 18 mo Prognostic: under 2yo: fever, large abdominal mass, wt loss, disseminated - blueberry muffin baby Older children: metastatic sx - bone pain, respiratory sx, GI complaints, periorbital region Produce catecholamines - elevated urine VMA and HVA Long term survival 90% in low to intermediate, 50% in high risk groups

Question 42

Wilms tumor

Answer 42

most common childhood renal tumor 2-5 yo, 95% before 10 risk: WAGR syndrome - wilms tumor, aniridia, genital anomalies, mental retardation -11p13 deletion Denys-Crash syndrome: gonadal dysgenesis - male pseudohermaphroditism -early nephropathy - diffuse mesangial sclerosis WT1 missense mutation - 11p13 ``` Beckwith-Wiedeman syndrome: Enlargement of body organs Macroglossia Hemihypertrophy Omphalocele Abnl large adrenal cortex WT2 - 11p15.5 ``` Presents as large abdominal mass, hematuria, pain in abdomen after trauma, intestinal obstruction, htn, pulmonary metastasis