Newman High Yield Flashcards
Change in oxygen dissociation curve in anemia
shifts to right
affinity of Hb for O2 reduced in tissues needing to be oxygenated
2,3-DPG increased within RBCs
EPO in anemia
levels go up, RBC production in BM increases
MCV and peripheral smear
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
Flow murmur
with anemia, cardiac output increases
get systolic ejection murmur
Intravascular hemolysis
Causes: mechanical injury complement fixation - autoimmune Intracellular parasites Toxins
Features: anemia *hemoglobinuria *hemoglobinemia *hemosiderinuria jaundice
Extravascular hemolysis
macrophages in liver and spleen eat RBCs
–> abnl abc membrane/deformilities of RBCs
Features:
*splenomegaly
anemia
jaundice
Reticulocyte count in anemia
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
Iron Deficiency Anemia
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
Lead toxicity anemia
microcytic, hypochromic
higher RDW - 17 or 18
low reticulocyte count
basophilic stippling
may have concurrent Fe deficiency
Thalassemias
Microcytic anemia
Mediterranean
Autosomal co-dominant disorders
Isoimmunization
positive direct coombs test
elevated indirect bilirubin
normocytic anemia
elevated reticulocyte count
ABO incompatibility
Rh incompatibility
Congenital hemolytic anemia
hemolysis
low enzyme activity
Smear: poikilocytosis, reticulocytosis, Heinz bodies, bite cells (G6PD def), spur cells (pyruvate kinase deficiency)
Congenital infection anemia
normocytic anemia
Low reticulocyte count
Parvovirus B19 - anaplastic anemia
HIV, syphilis, rubella, sepsis
Diamond Blackfan syndrome
macrocytic anemia with low reticulocyte count
Congenital pure red cell aplasia
Fanconi anemia
microcytic anemia and reticulocytopenia, thrombocytopenia, or leukopenia
pancytopenia - BM failure syndrome
GU and skeletal abnormalities
increased Chr fragility
*Mentzer index
MCV/RBC
less than 13 - thalassemia
>13 - iron deficiency
G6PD deficiency
episodic hemolysis
fava beans trigger
x linked recessive
Hereditary spherocytosis
AD in 75%
Heinz bodies and bite cells
Sickle cell disease
AR
Holly-Jolly bodies
Pernicious anemia
IF in parietal cells attacked by Ab
*Calculate ANC
((%neutrophils + % bands) x (WBC))/100
Severe ANC less than 500/uL - risk of serious infection
Cyclic neutropenia
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
Shwachman-Diamond syndrome
triad: neutropenia, exocrine pancreas insufficiency, skeletal abnormalities
AR
increased risk for myelodysplastic syndrome or leukemia
Leukocyte adhesion deficiency
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
Chronic granulomatous disease
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
Infection associated neutropenia
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…
Drug induced neutropenia
cytotoxic agents for treatment of malignancies
routine meds - abx, antiinflammatory, psychotropic drugs
Leukocytosis
usually Reactive process to infection
increased WBC, increased band cells
can be d/t chronic inflammation
Eosinophilia
hypersensitivity reactions, parasitic infections
congenital - AD, rare
Acquired: acute leukemia, MDS; 2ndary: infection, drugs, allergic disorders, autoimmune, malignancy, endocrinopathies
Hodgkin Lymphoma
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
Non-Hodgkin lymphoma
60% lymphomas in children and adolescents
Predisposing conditions:
SCID
Wiscott-Aldrich syndrome - X linked recessive
-Triad: Recurrent sinopulmonary infections, eczema, bleeding (2ndary thrombocytopenia)
Burkett lymphoma
NHL
abdominal (sporadic)
Head and neck dz (endemic) with involvement of bone marrow or CNS
Pathogenesis of leukemia
malignant transformation and clonal expansion of hematopoietic stem cells at early stage differentiation - unable to mature
Acute leukemia
97% of all childhood leukemias
CBC: initially high WBC –> marrow failure resulting in decreased WBC
Blasts in marrow
ALL
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
AML
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)
Quality of lymph nodes
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
Cat Scratch disease - eye involvement
parinaud’s syndrome: conjunctivitis, pre auricular adenines, conjunctival granuloma
neuroretinitis
Painless testicular mass
w/out hx of trauma/injury get a CXR to check for likely malignancy
Teratoma
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
Neuroblastoma
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
Wilms tumor
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
