Newman And Tyler Hl Flashcards
Anc
(%neutrophils+%bands)xWBC)/100
ANC<500
Risk of serious infection is high
Moderate neutropenia
500-1000
Mild neutropenia
1000-1500
Kostmann
Severe congenital neutropenia
Life threatening pyogenic infections, often in infancy
Why kostmann
Impaired myeloid differentiation caused by maturational arrest of neutrophil precursors
Inheritance kostmann
AR
Prob with kostmann
ANC<200, risk for AML
Cyclic neutropenia
Cyclic dever, oral ulcers, gingivitis, periodontal disease, recurrent bacterial infections
Defect cyclic neutropenia
Stem cell regulatory defect resulting in defective maturation
Sporadic or AD
Severe neutropenia <200 for 3-7 days fever every 3 weeks
CYCLIC
Malignancy from cyclic neutropenia
No
How is cyclic neutropenia missed
Bounce er ot er not same person
Schwachman diamond syndrome
Triad of neutropenia, exocrine pancreas, insuffiency and skeletal abnormalities
Defect schwachman diamond
Neutrophil mobility migration and chemotaxis in addition to neutropenia
What see with exocrine pancreas prob
Poor abrospion nutrients loose oily stool
Inheritance schwachman diamond
AR
Risk malignancy schwachman diamond
MDS or leukemia
Faconi
Bone marrow failure syndrome all cell lines effects
GU and skeletal abnormalities
Increased chromosome fragility
Inheritance faconi
AR present at 1-
Risk faconi
All cell lines effects
Pancytopenia bone marrow failure/aplastic
Risk AML brain tumor wilms tumor
Pancytopenia in kids
Faconi
Leukocyte adhesion defiency
Delayed separation of umbilical cord >3 weeks, recurrent and severe bacterial and fungal infections without pus accumulation
Cause of leukocyte adhesion defiency
Neutrophils have dismissed adhesion to surfaces
Inheritance leukocyte adhesion defiency
Very rare
AR
Jones syndrome hyper IgE
Severe eczema
Recurrent bacterial infections of skin
Recurrent pulmonary infections
IgE eosinophil up risk of HL
Chefiam higashi
Partial oculocutaneous albinism , peripheral and cranial neuropathies, neutropenia, recurrent pyogenic infections
Defects in granule morphogenetic, chemotaxis and degranulation, ineffective granulopoiesus
Rare AR
Chronic granulomatous disease
Recurrent purulent infections with fungal or bacterial catalase positive organisms, usually starting in infancy, chronic inflammatory granulomas
Defect chronic granulomatous disease
Defect in oxidative metabolism, absent generation of superoxide (which is toxic to microbe)
Inheritance chronic granulomatous disease
1/250000
Good prognosis
X linked recessive ar
Infection associated neutropenia
Many viruses cause neutropenia withint he first 2-3 days of illness lasting up to a week
Parvovirus B19 (5th slapped cheek appearance 1-1 weeks after injection with the virus)
Drug induced neutropenia
Cytotoxic agents for treatment of malignancies NOTORIOUS
When see neutropenia
Neutropenia from defiency
B12 vitamin
Symptoms vitamin b12 defiency
Glossitis cheliosis
Leukocytosis
Usually a reactive process to an infection which subsides with resolution of the acute event (viral or bacterial infection)
Chronic inflammation with leukocytosis
Auto immune disease
Oncológica leukocytosis
Yes!
Bad symptom of leukocytosis
Seizure
If someone has seizure and has high WBC
Not infection. Seizure causes demargination
Eosinophilia
Parasites
Leading cause of death by disease in kids BY DISEASE
Cancer
Most common cancer kids
Leukemia
15-19 lymphoma
Lymphoma
Most common cancer in adolescents
14-19
HL
Most common malignancy in the age 15-19
EBV
Pathogenesis HL
Reed sternberg cell
-pathognomic feature of HL
A large cell with multiple or multilobulated nuclei
Clincial HL
Unexplained fever 39
We loss>10% total body weight over 6 months
Drenching night sweated
Diagnose HL
Any patient with persisten unexplained LAD unassociated with an obvious underlying inflammatory or infectious process should undergo chest radiography to rule out a mediastinal mass before undergoing lymph node biopsy
Non hodgkin lymphoam
60% lymphoma in kid and adolescents
SCID
NHL secondary to SCID
Wish ot Aldrich syndrome x linked recessive NHL secondary to it
Recurrent sinopulmonary and ear infections
Severe atopic dermatitis
Bleeding
Burkitt lymphoma
Commonly manifests as abdominal (sporadic type) or head and neck disease (endemic type) with involvement of the bone marrow or CNS
Prognosis NHL
Patients with localized disease 90-1005 survival
Advanced 60-90% survival
-depends on pathological subtype, tumor burden of diagnosis as reflected in serum lactate DH LDH levels, presence or absence of CNS disease and specific sites of metastatic
Childhood leukemia
Most common under 15
And brain tumor
Leukemia pathogenesis
Malignant transformationa nd clincal expansion of HSC at an early stage of differentiation hat are then unable to undergo further maturation
Lymphoblastic leukemia
Myeloid
Acute Leukemias -97%
ALL AML genetics
Trisomy 21 both
Supraclavisular lymph node
Not common so be aware
Perk up ears
Most important work up LAD
H AND P
SMOOTH MOBILE
OK
Matted down ruff and in place not expect
Perk up
Are you warm fever systemically ill
PE node
> 2 cm enlarged
Location important supraclavicular not normal!
Tendernesss, erythema, swelling, distal lesions, spleen liver size
Unusual spot node
Supraclavicular
Age and node
Not palpable in babies
Maybe shorty with viral illness
Lymphoma rare under 10
Reactive cervical LAD very common prescrhool . Early school age
Reactive cervical LAD
Really common
Kid with conjunctivitis if have preauricular node increase chance bacterial
> 2 cm
May be significat but put in context with patient
Watchful waiting
If etiology obvious or if nodes not concerning
Antibiotic
Bacterial fever
Shotgun approach
NO
When biopsy node
No go away or gets bigger
Kawasaki
Unilateral cervical node fever 102 for 5 days
Cat scratch
Bartonella
Axilla nods six with low grade fever no other kids sick. Sore left hand healed .
Bartonella
Treat cat scratch disease
Azithromycin
Self limited but treat
Eye cat scratch disease
Neuroretinitis
Bartonella
Cat scratch disease isolated LAD often in axilla or in neck
14 year old boy lump chest apprehensive supraclavicular , and lump on privates, really tired , cough a lot, nose bleeds
CHEST X ray
See radioopaque mass in anterior cavity
Lymphoma to oncologist can metastasize to testicle
Testicular mass painless
Likely malignancy
Ill kid supraclavicular node coughing fatigue and
So more work up
Tumor like lesions
Heterotopic and hamartoma
Heterotopic
Microscopically normal cells in abnormal spots
Hamartoma
Overgrowth of native to organ ins high masses
Hemangioma
Most common tumor of infancy
Port wine-do CT scan
Hemangioma and thrombocytopenia
Huh
Lymphatic tumor
Lymphangioma
Fibrous tumros
Ok
Teratomas
Well differentiated cystic lesions
Most common solid tumor
Wilms
Neuroblastoma
Test when see blueberry muffin big mass
Less than 2 yo usual presentation is fever, large abdominal mass, blueberry muffin rash
Neuroblastoma test
VMA HVA
Wilms
1/10000
Most common primary renal tumor of childhood 204 yo
See big abdominal mass
Wilms tumor
Beckwith wiedemann
Enlarge body organs Macroglossia Hemihypertrophy****** Omphalophcele Abnormal large cells in adrenal
11p15.5 wt2
Wilms tumor
Large abdominal mass
Anemia
Reduced rbc volume or hbg content
Anemia shift
Right less affinity for oxygen
Easier to dump off
2,3 DPG increase in anemia
Increases within the RBC
Oxygen dissociation curve shift right
Work of anemic
H and p
Family history
Anemia, splenomegaly, jaundice, eagerly onset gallstones
Clincial features kids anemia
Pallor, sleepy, irritability, decreased exercise tolerance, flow murmur
Gall stones and jaundice
Hemolysis
2-3 have gallstones?
Not really
Flow murmur anemia
Increase CO to get oxygen to organs
Anytime increase CO
Get flow murmur doesn’t mean not right
Anemia severe symptoms
Weak, tachypnea, SOB, tachycardia, cardiac dilation/cardiomyopathy
Slower developing anemia
Slower dev better can compensate
Hemoglobin
Actual amount of hemoglobin in blood
Hematocrit
Volume percentage of rbc in blood
H/H
Hemoglobin/hematocrit
RDW up
Increase variation in side of rbc
In iron defiency anemia
MHCH
Mean corpuscular hemoglobin concentration
Grams of hbg per 100 ml
MCH
Mean corpuscular
Average content of HGB per RBC
Picograms per cell
RDW
Variability to sizes
RDW
Anisocytosis
Peripheral smear
Anisocytosis, poikilocytosis
Schistocytes is-fragments (hemolysis)
Decreased RBC production in bone marrow differential
Ineffective erythropoietin
Complete or relative failure of erythrocytosis
Increased destruction of RBC differential
Hemolysis
Intravascular extravascular
Anemia, hemoglobinuria, hemoglobinuria, hemosideria
Extravascular
Reticuloendothelial system which is everywhere
Espicially liver spleen phagocytosis rbc can be due to rbc membrane abnormalities or antibodies on them
See splenomegaly, anemia, jaundice
Bleeding anemia
Acute
- hemorrhage
- normochromic normocytic
Chronic
-hypochromic microcytic bc of iron defiency
Reticulocyte count
Low or low normal number of reticulocytes in a patient with anemia is indicative of an inadequate bone marrow response
Increased number of reticulocytes in a normal bone marrow response to anemia
It’s trying to keep up
Bone marrow respongin appropriately
Low normal reticulocyte count is an inadequate bone marrow response
In face of significant anemia
Not appropriate inadequate response
If think bone marrow failure
Bone marrow aspirate
Hematocrit hemoglobin relationship normally
Hemoglobin 12
Hematocrit 3x 36
Reticulocyte low
Bone marrow not enough building blocks
Up RDW and reticulocyte count up
RDW iron defiency
Anemic MCV normal reticulocyte count low rdw normal
Transient erythroblastopenia see pop up
Objectives know where they fall on graph
Ok
Diamond black fan
Pure rbc aplasia
Macrocytic
Decreased reticulocytes
Increase reticulocytes (bone marrow doing what do) what do
Do direct antibodies test for antibodies on rbc
newborn anemia peripheral smear then what
Blood loss
Peripheral smear abnormal new born anemia
Hemolysis!
Multiple cell lines affected
Think back ot bone marrow
At 2 months
Kids physiologic nater for hemoglobin nothing wrong but do h and H and hemoglobin 10 or 9 and everything else ok just physiologic nater
Kids born with a lot of rbc with short lifespan lot hemolize as bone marro pick up