Dr. Newmann - Red/White Cell Disorders Pediatrics Flashcards
When does the infant have the lowest amount of hemoglobin
2mos
2,3-DPG concentration does what to O2 distribution curve
Shifted to the right (lower affinity of O2, since tissues signal they need O2)
Anemia does what to the heart
Increase CO
Murmur can be heard due to low viscosity
history that can point to anemia
Infection , travel, exposures, chronic illness, anemia in family, splenomegally, jaundice, early onset gallstones
Anemia SX that are common to see on someone
Pale, irritability, sleepiness, low exercise tolerance, bloody stools, LAD, fever, viral Sx, heavy menses, SOB, cough
Diamond-Blackfan Syndrome
Congenital only RBC aplasia
- presents in infancy (erythroid precursor apoptosis)
- low reticular count
- macrocytic anemia
Fanconi Anemia
Inherited aplastic anemia (most common)**
- macrocytic anemia
- LOW reticular count
- leukopenia + thrombocytopenia (low RBC, plt, WBC)= apoptosis in BM
- usually young up to 10yo when DX**
- can become panocytopenia
IDA in pediatric patients
- usually from not eating enough Fe
- microcytic, hypochromic high RDW
- HIGH transferrin, LOW ferritin and FE sat
- SX : pale, and big cow milk drinker (you get Fe from mothers breast milk)**
Mentzer Index
When pt had mild microcytic anemia : MCV/RBC
- <13 = IDA
- > 13 = B-thalassemia trait
- = 13 = indeterminate
How to calculate ANC (Absolute Neutrophil Count)
ANC = (%N +%Bands) (WBC) / 100
Mild : 1000-1500
Moderate : 500-1000
Severe neutropenia : 500
What drug causes Neutropenia
Chemotherapy
Kostmann Syndrome
(Severe Congenital Neutropenia) AR
- Life threatening pyogenic infections early in life
- Impaired myeloid differentiation caused by maturation arrest of N precursors
- ANC <200
- high risk of AML
Cyclic Neutropenia
Cyclic Fever, oral ulcers, gingivitis, periodontal disease, bacterial infections **
- stem cell regulatory defect resulting in defective maturation **
- ANC <200 for 3-7 days every 15-35 days **
(NO RISK for any AML)**
Schwachman- Diamond Syndrome
TRIAD**
1. Neutropenia
2. Exocrine pancreas insufficiency
3. skeletal abnormalities
= X N mobility, migration, chemotaxis, count
- (higher risk to get MDS or other leukemia)
Fanconi Anemia SX and when do you usually see it, and in risk of what
BM cant make any cells lines normally (aplastic anemia) = can become panocytopenia
- GU + Skeletal anomalies, increased chr fragility
- First 10 years of life
- AML, Brain tumors, Wilms tumor
Leukocyte adhesion deficiency
SX
Why
Risks
Rare
- Delayed separation of umbilical cord stump (3weeks)**
- bacteria and fungal infections with NO PUS**
- poor wound healing
- N can adhere to cross BV
- No risks
Hyper-immunoglobulin E syndrome (Job syndrome)
What
Why
Risk
- TRIAD **
1. Severe eczema
2. Recurrent staph skin infection (or other bacteria)
3. Recurrent pulmonary infections (bacteria/fungal) - no N chemotaxis and high IgE
- Hodgkin lymphoma risk
Chediak-Higashi Syndrome
SX
Why
Risks
- Oculocutaneous albinism, neuropathies, recurrent pyogenic infections
- No N chemotaxis/degranulation, X granulopoisis (die dry age 20yo)LYSOSOME BREAKDOWN PROBLEM
- No risks
Chronic granulomatous disease
SX
Why
Risks
- Recurrent purulent infections (fungal/bacterial), CATALASE +
(Usually infancy, chronic inflammatory granulomas) - X oxidative metabolism, X superoxide in N
(X-linked) - No risks
Parovirus B19
5th disease of childhood
- bright red cheeks, diffuse lacy red rash)
Anemia/ ( can cause thrombocytopenia +neutropenia), I think aplastic anemia
Digeorges Syndrome
What is it and causes what
X thymus
Causes T-cell immunodeficiency
Leading cause of death in infancy in US
Leading cause of death in due to disease
- Unintentional injuries (accidents)
2. Brain Cancer and Leukemia
Most common malignancy in 15-19yo and risk factors to this
- Hodgkin Lymphoma
2. HHV-6, cytomegalovirus, EBV
B signs for Hodgkin’s lymphoma
- Unexplained fever (102.2F, 39C)
- Wt loss > 10% over 6 months
- Drenching night sweats
When should I do a chest X-ray in a pt with lymphadenopathy
- Any pt with unexplained LA, with no association to inflammation or infection process (rule out mediastinal mass*)
- Any pt with persistent LAD and with cough, SOB, CP
- Pt with lymphoma
Most common type of lymphoma in children and adolescents
Non-Hodgkin lymphoma
Wiscott-Aldrich syndrome
- X-linked**
- TRIAD**
1. Ear infections/ sino-pulmonary infections
2. Severe atopic dermatitis
3. Thrombocytopenia (bleeding) - RISK TO GET NON-HODGKIN LYMPHOMA
3 types of Burkitts lymphoma
- Sporadic : abd disease, EBV, Africa
- Endemic Type : head,neck, Africa, EBV/ Malaria
- Immunodeficiency- restated: immunosuppressive drug use
Number 1 malignancy in children is what
Leukemia (ALL is 97%)
Which genetic condition can one be born with that is associated with AML and ALL
Trisomy 21
Plt counts normal
Severe
Life threatening
150,000-450,000
20,000
10,000
Decreased Plt production caused by what 3 causes
- VIT B12, folate, FE deficiency
- BM problem : AA, leukemia, lymphoma
- Genetic problem : Wisconsin-Aldrich, Fanconi, schwachman-diamond, thrombocytopenia-absent-radius syndrome (TAR)
Causes for increased plt destruction and consumption
- ITP
- HUS/DIC
- TTP
- Kasabach-Merritt phenomenon
Reasons for plt sequestration
Hypersplenism
Von-Willebrand disease
ITP
Immune thrombocytopenia purpura
ANTI-plt antibodies
(50% filled by viral infection lasting 1-3 weeks)
<20,000plt
- normal PT and PTT
- normal other cell lines (otherwise check BM)
SX : usually normal, bleeding
Kasabach- Merritt phenomenal
Thrombocytopenia + hypo-fibrinogenemia + GIANT HEMANGIOMAS + intravascular coagulation
3 red flag sx in thrombocytopenia pt
- Panocytopenia sign
- Raised LAD (cancer can be it)
- Renal impairment
Acute vs chronic ITP
ACUTE
ACUTE ITP : 2-6yo 1-3 week infection before <20,000 plt High lymphocytes 80% spontaneous remission (2-6 weeks)
Acute vs chronic ITP
Chronic
20-40yo Female No infection before 30,000 - 80,000plt Low lymphocytes Mo-years (no spontaneous remission usually)
HUS
Hemolytic uremic syndrome
- after acute gastroenteritis
- TRIAD
1. Microangiopathic hemolytic anemia
2. Thrombocytopenia
3. Acute renal failure - blood in urine, if proteinuria then worse prognosis
Which organism is usually the cause of gastroenteritis cussing HUS
E. coli 0157:H7
