Anemias Flashcards
Four things that people with sickle cell trait are at increased risk for
VTE
Ckd
Renal medullary carcinoma
Hematuria
When does sickle cell trait start to have the risk of sudden-death with physical activity
Without a dehydration rhabdo or heat exposure
Two tests to detect sickle cell disease
Hemoglobin electrophoresis and peripheral smear
What deficiency do sickle cell disease patients have and what does expose them to
Folate for megaloblastic macrocytic anemia
Nine causes of vaso-occlusive crisis
Nothing hypoxia dehydration acidosis Fatigue cold-weather alcohol pregnancy emotional stress
Six associated symptoms with vaso-occlusive crisis
Fever swelling tenderness tachypnea HTN nausea and vomiting
How long do vaso-occlusive crisis last
2 to 7 days
How do you diagnose vaso-occlusive crisis
Clinically
How do you treat vaso-occlusive crisis
NSAIDs the narcotics
always hydrate
For indications to use hydroxyurea
More than three episodes a year
history of ACS
history of vaso-occlusive events
history of symptomatic anemia
The side effect of hydroxyurea
Suppress all lines
Presentation of ACS
Chest pain
fever
cough
low O2 sat
What is ACS
New infiltrate with the pulmonary symptoms in a patient with SCD
Three ways to get increased cycling in the pulmonary vascular in ACS
Fat embolism
pulmonary infarct
infection by pneumonia or mycoplasma
Four steps of treating ACS
Hydrate with caution
oxygen support
antibiotics
transfusion
Three neurologic complications of sickle cell disease
Stroke seizures narrow cognitive decline
How do we determine children’s risk for stroke and SCD
Transcranial Doppler
Describe aplastic crisis and SCD
Parovirus B 19 causes an infection of red blood cell progenitors in the marrow which impairs cellular division for a few days this causes a rapid drop in hemoglobin with the reticulocyte count less than one
How soon do aplastic crisis patients get better
7 to 10 days
When does splenic sequestration usually happen in sickle cell disease patients
Within the first five years but can occur anytime
What bacteria’s are especially harmful to kids with sickle cell disease
strep pneumo
H flu
Which bacteria most dangerous two adults with sickle cell disease
E. coli
Samonella
Strep pneumo
What to viruses or more virulent in SCD
Parovirus
H1n1
What cardiovascular event are SCD patients more prone to
Myocardial infarction because likely secondary increased 02 demand
Deficiency and hereditary spherocytosis
Spectrin
Three clinical manifestations of hhereditary spherocytosis
Anemia jaundice splenomegaly
Clinical picture of mild hereditary spero-cytosis
No anemia modest reticulocytosis little splenomegaly or Jandus not diagnosed until adulthood
When do you need regular transfusions and hereditary spherocytosis
When is severe
Seven key diagnostic features of hereditary spirit cytosis
Reticulocytosis microcytic anemia hyperchromic cells elevated RDW spherocytes on peripheral smear pseudohyperkalemia osmotic fragility test
For complications of hereditary spherocytes cytosis
Lego ulcers
hypertrophic cardiomyopathy
movement disorders
gallstones
Three treatments for hereditary spherocytosis
Folic acid
blood transfusions
splenectomy
Six causes of autoimmune hemolytic anemia warm
Idiopathic viral infection systemic lupus immune deficiency malignancy of the immune system drugs
Six drugs that cause warm hemolytic anemia
Sulfas cephalosporins rifampin ibuprofen penicillins fluroquinolones
Drug to use for a long-term treatment of warm autoimmune hemolytic anemia
Azothioprine(imuran)
What drug can’t you use and warm hemolytic anemia
Methotrexate
Is warm or cold hemolytic anemia more severe
Warm
Two most common infections that cause cold hemolytic anemia
Pneumonia
mono
Most common neoplasm to cause cold hemolytic anemia
Lymphoma
Is hereditary hemachromatosis dominant or recessive
Recessive
For places the iron deposits and hereditary hemachromatosis
Heart liver pituitary pancreas
Seven manifestations of hereditary hemachromatosis
Liver disease Hepatocellular carcinoma diabetes mellitus joint pain dilated cardiomyopathy hypogonadism skin changes
The only definitive test to diagnose hereditary hemachromatosis
Liver biopsy
When don’t we treat hereditary hemachromatosis
Ferritin less than 1000
asymptomatic
Best way to treat hereditary hemachromatosis if you have to
Phlebotomy
Most common population of sickle cell
Sub-Saharan Africans
Three populations most at risk for taco
Young old and surgical ICU settings
Best noninvasive test to determine total Iron body stores
Ferritin
Two things that affect TIBC
Pregnancy OCP
How much elemental iron does one need for supplementation a day
150 to 200 mg
How soon should hemoglobin be back to normal with treatment of iron deficiency anemia
6 to 8 weeks
What is often mistaken for a vitamin B12 deficiency
Sideriblastic anemia
Transferrin level in sideroblastic anemia
Low
RDW in sideroblastic anemia
High
Treatment for anemia of chronic disease if you must
EPO
Seven causes of a plastic anemia
Idiopathic cytotoxic radiation drugs viral immune disorder pregnancy Anorexia Dancing anemia
6 categories of clinical manifestations in aplastic anemia
Cardiopulmonary compromise fatigue recurring infection mucosal hemorrhage Pallor and petechiae rash no lymphadenopathy No hepatosplenomegaly
Four characteristics of sudden onset cute hemolytic anemia
Jaundice
Pallor
dark urine
with or without abdominal or back pain
Hemoglobin reaction to a cute hemolytic anemia
Hg fall of 3 to 4
Drugs that cause hemolytic Anemia in g6pd
Fluoroquinolones antimalarial's nitro sulfa drugs Tylenol aspirin methylene blue INH
What chains are excess in a thalassemia
Gamma in newborns
Beta on adults
Where is a thalassemia the most common
Southeast Asia
3 Severe lifelong problems in HbH disease
Microcytic hemolytic anemia
infective erythropoeisis
splenomegaly
What are four beta chains bound together that are soluble and formed from Alpha thalassemia intermedia
Hemoglobin h
What are four gamma chains bound together called
Hb Bart’s
What has a higher affinity for oxygen and then HBA but cannot release it
HB Barts
What is the MCV in a thalassemia and why?
Well because hemoglobin makes up 35% of the cell volume
What are target cells associated with
A thalassemia
Reason behind target sells
The KCL cotransporter is broken in a Palastine yeah and allows fluid to leak out causing dehydration
When do you see basophilic stippling
Hemolytic anemia and a and B thalassemia
Iron transferrin and ferritin levels in a thalassemia
All higo
Treatment for a thalassemia minima and minor
None
Treatment for h BH disease
Follow bloodwork support with transfusion avoid oxidative stress but no bone marrow transplant
What exacerbates hemolysis in HbH dz
Oxidation (same as g6pd)
What percent is hemoglobin H in Alpha thalassemia intermedia
5 to 30%
Is Alpha thalassemia intermedia symptomatic at birth
Yes with neonatal jaundice and anemia
What population is beta thalassemia common in
Mediterranean descent
Presentation of beta thalassemia minor
Asymptomatic mild anemic discovered incidentally
Presentation of Beta thalassemia intermedia
Varies
Synonym for beta thalassemia major
Cooley’s anemia
Eight characteristics of beta thalassemia major
Abdominal swelling growth retardation irritability Jaundice Pallor skeletal abnormalities splenomegaly life long transfusion dependent
What anemia gets an altered protein synthesis of spectrum in addition to their problems
Beta thalassemia
Which thalassemia has premature a pop ptosis
Beta
M HC and MCHC of beta thalassemia
Microcytic hypo chronic but some will be very dense