Newman High Yield Flashcards

1
Q

Change in oxygen dissociation curve in anemia

A

shifts to right

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

2,3-DPG increased within RBCs

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2
Q

EPO in anemia

A

levels go up, RBC production in BM increases

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3
Q

MCV and peripheral smear

A

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
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4
Q

Flow murmur

A

with anemia, cardiac output increases

get systolic ejection murmur

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5
Q

Intravascular hemolysis

A
Causes:
mechanical injury
complement fixation - autoimmune
Intracellular parasites
Toxins
Features:
anemia
*hemoglobinuria
*hemoglobinemia
*hemosiderinuria
jaundice
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6
Q

Extravascular hemolysis

A

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

Features:
*splenomegaly
anemia
jaundice

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7
Q

Reticulocyte count in anemia

A

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
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8
Q

Iron Deficiency Anemia

A
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

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9
Q

Lead toxicity anemia

A

microcytic, hypochromic
higher RDW - 17 or 18

low reticulocyte count
basophilic stippling

may have concurrent Fe deficiency

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10
Q

Thalassemias

A

Microcytic anemia

Mediterranean
Autosomal co-dominant disorders

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11
Q

Isoimmunization

A

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

ABO incompatibility
Rh incompatibility

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12
Q

Congenital hemolytic anemia

A

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

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13
Q

Congenital infection anemia

A

normocytic anemia
Low reticulocyte count

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

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14
Q

Diamond Blackfan syndrome

A

macrocytic anemia with low reticulocyte count

Congenital pure red cell aplasia

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15
Q

Fanconi anemia

A

microcytic anemia and reticulocytopenia, thrombocytopenia, or leukopenia

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

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16
Q

*Mentzer index

A

MCV/RBC

less than 13 - thalassemia
>13 - iron deficiency

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17
Q

G6PD deficiency

A

episodic hemolysis
fava beans trigger
x linked recessive

18
Q

Hereditary spherocytosis

A

AD in 75%

Heinz bodies and bite cells

19
Q

Sickle cell disease

A

AR

Holly-Jolly bodies

20
Q

Pernicious anemia

A

IF in parietal cells attacked by Ab

21
Q

*Calculate ANC

A

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

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

22
Q

Cyclic neutropenia

A

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

23
Q

Shwachman-Diamond syndrome

A

triad: neutropenia, exocrine pancreas insufficiency, skeletal abnormalities

AR

increased risk for myelodysplastic syndrome or leukemia

24
Q

Leukocyte adhesion deficiency

A

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

25
Q

Chronic granulomatous disease

A

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

26
Q

Infection associated neutropenia

A

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…

27
Q

Drug induced neutropenia

A

cytotoxic agents for treatment of malignancies

routine meds - abx, antiinflammatory, psychotropic drugs

28
Q

Leukocytosis

A

usually Reactive process to infection

increased WBC, increased band cells

can be d/t chronic inflammation

29
Q

Eosinophilia

A

hypersensitivity reactions, parasitic infections

congenital - AD, rare

Acquired: acute leukemia, MDS; 2ndary: infection, drugs, allergic disorders, autoimmune, malignancy, endocrinopathies

30
Q

Hodgkin Lymphoma

A

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

31
Q

Non-Hodgkin lymphoma

A

60% lymphomas in children and adolescents

Predisposing conditions:
SCID
Wiscott-Aldrich syndrome - X linked recessive
-Triad: Recurrent sinopulmonary infections, eczema, bleeding (2ndary thrombocytopenia)

32
Q

Burkett lymphoma

A

NHL

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

33
Q

Pathogenesis of leukemia

A

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

34
Q

Acute leukemia

A

97% of all childhood leukemias

CBC: initially high WBC –> marrow failure resulting in decreased WBC

Blasts in marrow

35
Q

ALL

A

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

36
Q

AML

A

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)

37
Q

Quality of lymph nodes

A

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

38
Q

Cat Scratch disease - eye involvement

A

parinaud’s syndrome: conjunctivitis, pre auricular adenines, conjunctival granuloma

neuroretinitis

39
Q

Painless testicular mass

A

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

40
Q

Teratoma

A

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

41
Q

Neuroblastoma

A

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

42
Q

Wilms tumor

A

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