RBC Disorders Flashcards by plasma opp

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

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Two types of Hemolytic Anemia

1) Intravascular

2) Extravascular

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General Presentation of Intravascular hemolytic anemia

1) Hemoglobinuria
2) Hemoglobinuria
3) Hemosiderinuria

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General Presentation of Extravascular Hemolytic Anemia

1) Splenomegaly
2) Jaundice
3) Anemia

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Howell Jolly Bodies in Hereditary Spherocytosis is due to

DNA Inclusions

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How do you Treat Hereditary Spherocytosis. Does that get rid of HS?

Splenectomy

No red blood cells are still spherocytic w/ Howell Jolly Bodies

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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

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Triggers of glucose 6 Phosphate Dehydrogenase Def.

1) Fava Beans
2) Drug
3) Stress
4) Infection

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Clinical Feature of G6PD Def.

1) Antimalarials
2) Sulfanamides
3) Nitrofurantiona

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Heinz Bodies are characteristic of

G6PD Def.

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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

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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

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Diagnosis of B-Thalassemia

Hemoglobin Electrophoresis: Increased HbF and HbA2

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X-ray show ___ in B-thalassemia

Crew cut appearance due to medullary and extramedullary hematopoiesis

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What is a major cause of death in Paroxysmal Nocturnal Hemoglobinuria

Thromboembolism: Hepatic, Portal, and Cerebral Veins

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Gene mutation in PNH

PIGA mutation

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Is PNH intravascular or extravascular

Intravascular

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Complications of PNH

1) Iron Def, Anemia due to hemoglobinuria

2) AML (develops in 10% pts)

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Treatment of PNH

Eculizumab: Blocks C5–> C5a

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Treatment Side Effect of PNH

1) Eculizumab

- Sus, to neisseria meningiditis

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Methotrexate causes what def.

Folate Def.

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Why does Methotrexate cause a Folate Def.

1) Inhibits DHF Reductase

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Causes for a Lack of Folate

1) Decrease Intake
2) Impaired Absorption
3) Blocked Metabolism
4) Increased requirements

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Clinical Presentations of Folate Def,

1) Neural Tube Defects
2) Mental retardation
3) Anemia

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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

RBC Disorders Flashcards

(77 cards)