RBC Disorders Flashcards
Describe why a patient with chronic anemia will present with subtle symptoms of anemia vs a patient with acute blood loss
1) Body has time to compensate
Two types of Hemolytic Anemia
1) Intravascular
2) Extravascular
General Presentation of Intravascular hemolytic anemia
1) Hemoglobinuria
2) Hemoglobinuria
3) Hemosiderinuria
General Presentation of Extravascular Hemolytic Anemia
1) Splenomegaly
2) Jaundice
3) Anemia
Howell Jolly Bodies in Hereditary Spherocytosis is due to
DNA Inclusions
How do you Treat Hereditary Spherocytosis. Does that get rid of HS?
Splenectomy
No red blood cells are still spherocytic w/ Howell Jolly Bodies
Pt with Hereditary Spherocytosis comes in and presents with severe Anemia. What microbe do you suspect that this pt has?
1) Parvovirus B19
- Infects precursor red blood cells
Triggers of glucose 6 Phosphate Dehydrogenase Def.
1) Fava Beans
2) Drug
3) Stress
4) Infection
Clinical Feature of G6PD Def.
1) Antimalarials
2) Sulfanamides
3) Nitrofurantiona
Heinz Bodies are characteristic of
G6PD Def.
Clinical Presentation of Sickle Cell Anemia
1) Acute Respiratory Distress and Acute Chest Syndrome
2) Autosplenectomy and infection (encapsulated bacteria)
3) Aseptic Bone Necrosis and Osteomyelitis
How does iron overload occur in B-Thalassemia Major
1) Ineffective erythropoiesis and hemolysis
2) Tissues become Hypoxic
3) Increase EPO release
4) Marrow expansion and skeletal deformities
Hepcidin Suppression –> Iron Overload
Diagnosis of B-Thalassemia
Hemoglobin Electrophoresis: Increased HbF and HbA2
X-ray show ___ in B-thalassemia
Crew cut appearance due to medullary and extramedullary hematopoiesis
What is a major cause of death in Paroxysmal Nocturnal Hemoglobinuria
Thromboembolism: Hepatic, Portal, and Cerebral Veins
Gene mutation in PNH
PIGA mutation
Is PNH intravascular or extravascular
Intravascular
Complications of PNH
1) Iron Def, Anemia due to hemoglobinuria
2) AML (develops in 10% pts)
Treatment of PNH
Eculizumab: Blocks C5–> C5a
Treatment Side Effect of PNH
1) Eculizumab
- Sus, to neisseria meningiditis
Methotrexate causes what def.
Folate Def.
Why does Methotrexate cause a Folate Def.
1) Inhibits DHF Reductase
Causes for a Lack of Folate
1) Decrease Intake
2) Impaired Absorption
3) Blocked Metabolism
4) Increased requirements
Clinical Presentations of Folate Def,
1) Neural Tube Defects
2) Mental retardation
3) Anemia
Diagnostic of Folate Def.
Increased levels of Homocysteine
Pathogenesis of Aplastic Anemia
genetic altered stem cells
What is the rare inherited form of Aplastic Anemia
Faconi Anemia
Causes of Pure Red Cell Aplasia
1) Autoimmune (Thyoma)
2) Parvovirus B19
Leukoeryhtroblastosis is characteristic of …..
Myelophthisic Anemia
What is Myelophthisic Anemia
Space-occupying in marrow replace normal hematopoietic cell
List the values that are tested for in Hemolysis labs
1) Lactate Dehydrogenase (LDH)
2) Indirect (unconjugated) Bilirubin
3) Haptoglobin: Plasma protein that binds to free hemoglobin
Male Pts or postmenopausal females w/ iron def. anemia you should check for ___
GI bleed
-Colonoscopy
How do you treat iron def. anemia
Underlying disease
what are the symptoms of Hypovolemic Shock
1) Hypotensive
2) Tachycardia
3) Weak Pulses
3 Consequences of Acute Blood Loss
1) Hypovolemia
2) Myocardial ischemia
3) renal Failure
Is the blood loss compensated or uncompensated in acute blood loss
Uncompensated
- Normocytic Anemia
- Normal retic count
Treatment for Hypovolemic Shock
1) Apply pressure/compression
2) Obtain IV access (two large bore IVs)
3) Rapid Infusion of 1L NS or LR
4) Blood
Acute Blood Loss scenario will wither be ___ or ___
1) Hypovolemia
2) Heart Attack
Know child has SCD. What is something you can do to prevent stroke from occurring
1) Blood Transfusions during childhood
Sx of Acute Chest Syndrome
1) Chest Pain
2) Dyspnea
3) Cough
4) Hypoxemia and Fever
CXR will show what when a pt has Acute Chest SSyndrome
Pulmonary infiltrate
Pathogenesis of avascular Necrosis
Microvascular occlusions
- causes painful crises
Fever + Hip pain is ____ until proven otherwise
Osteomyelitis
How to prevent painful attacks w/o meds in SCD
1) Avoid High Altitudes
2) Avoid Strenuous Exercise
Pt who has anemia and High MCV but Normal RDW is indicative of
1) Chemotherapy
2) Antivirals
3) Alcohols
4) Aplastic Anemia
Treatment of Aplastic Anemia
1) Transfusion Support
2) Growth Factor Support
What are the ways that iron can be delivered for pts with iron def. anemia
1) Oral Iron:
2) Parentraral (Colloidal) Iron
Dosage iron to a iron def. pt.
1) 200-400 mg FERROUS iron/day in a SINGLE dose
Oral Iron cannot be ___ or ___
1) Enteric Coated
2) Sustained-release
Oral iron cannot be taken with
1) Water
2) Juice
Indications for Iron given via IV
1) Iron Malabsorption
2) Intolerance of oral therapy
3) Noncompliance
What is the least expensive drug that is give for iron via IV
LMW Dextran
- Others are: Sodium Ferric gluconate complex, iron sucrose, iron isomaltoside
Side Effects of Iron supplementation
1) Nausea
2) Constipation
3) Anonexia
4) Heart Burn
5) V/D
6) Dark Stools
Most common population involved with iron poisoning
1) Young Children
Sx of Iron Poisonings
1) Necrotizing Gastroenteritis w/ vomiting
2) Abdominal Pain and bloody diarrhea –> Shock
Iron Poising can eventually lead to __, __, and __ in young children
1) shock
2) lethergy
3) Dyspnea
Treatment for Iron Poisonings
Deferoxamine (Iron chelating Compound)
When would you treat a Vit B12 def with Cyanocobalamin via IV
1) Neurological Symptoms Present
Dosage for Oral Vit B12
1) 1-2mg/day for 2 wks
2) then 1mg daily
Treatment/Dosage for Folate Def.
1) Oral Folate
2) 1mg/day for 4 months
Treatment/Dosage for Folate Def. w/ malabsorption present
1) 1-5mg/day
The physiologic lowest point for hemoglobin occurs at ___ of age and = to 10.1-14.5
2mo
How does anemia affect hemoglobin-oxygen-dissociation curve
1) 2,3 DPG increase due to hypoxia and anemia
2) Shifts Curve to the Right
Signs of Anemia in a Child
1) Tired
2) Irritable
3) Decrease Exercise Tolerance
4) Pale
5) blood in stool/urine
6) Increased LAD
7) SOB
8) Fever
Main causes of anemia
1) Decreased Production
2) Increased Destruction
3) Blood Loss: Acute or Chronic
Diamond- Blackfan Syndrome is ….
Red Cell Aplasia
Pathophysiology of Diamond-Blackfan Syndrome
1) Increased Apoptosis of erythroid Precursors
What type of anemia is Diamond-Blackfan Syndrome (MCV)
1) Macrocytic Anemia w/ low to normal retic count
What is the most common inhereted Aplastic anemai
1) Fanconi anemia
Pathophysiology of Fanconi Anemia
Increased Apoptosis in bone marrow
When does Fanconi Anemia present
Up to 10yrs
MDS is a precursor to ____
AML
**Esp if MDS is due to prior cytoxic therapy/radiation (t-MDS)
___ is the clonal disorder w/ morphologic manifestations of many cell lines
Myelodysplastic Disorder
How would you distinguish a MDS?
1) Dysmorphic Features in one or more lineages
2) Chromosomal Analysis
3) Blast Count
Describe the types of MDS in terms of blast count.
1) RAEB-1: 5-9%
2) RAEB 2: 10-19% (ANEMIA will probabl be present at this point)
List the Histomorphologic Features of MDS
1) Dyserythropiesis
2) Ring Sideroblasts
3) Pseudo Pelger-Huet Cells
4) Dysmegakaryopoiesis
List the diver mutations in MDS
1) Epigenetic Mutations
2) RNA splicing factor mutations
3) Transcription factor mutations
