Hematopathology- Schoenwald Flashcards
RBC disorders: Describe the very basic pathology of anemia.
A decrease in RBCs
RBC disorders: What are the three mechanisms of anemia?
- Blood loss (acute or chronic)
- Decreased production of RBC
- Increased destruction of RBC
RBC disorders: Describe hemolysis
Destruction of RBCs
RBC disorders: How do you evaluate for anemia?
Size and hemoglobin concentration (MCV and MCHC)
Classification of anemias: In blood loss (acute) what will you see on evaluation of the RBCs? (initially and later).
- Initially: normocytic normochromic
- Later: Hypochromic, reticulocytosis
Ex’s of blood loss (acute) that can cause anemia? (list 3)
- Acute volume depletion.
- RBC reduction due to fluid influx
- Rapid RBC regeneration
classification of anemias: RBCs of blood loss (chronic)?
Hypochromic (means the RBC’s have less Hgb than normal)
Classification of anemias: Characteristics of blood loss (chronic)?
Reduced Iron stores
lassification of anemias: What types of anemia are under the category of increased destruction?
- Hemolytic anemias
- Immunologic
- Mechanical
- Hereditary
Classification of anemias: Characteristics of hemolytic anemias?
- Features of hemolysis
- Splenomegaly
Classification of anemias: Characteristics of immunologic anemias?
- Autoantibodies against RBC
Lab finding (pertaining to cells) associated with immunologic anemias?
- Reticulocytosis
Classification of anemias: RBCs of Mechanical anemias?
Shistocytes
classification of anemias: examples of mechanical anemias?
- HUS (hemolytic uremic syndrome)
- TTP (thrombocytopenic purpura)
- Trauma-valve
Classification of anemias: RBC (types) of hereditary anemias? (list 5)
- Spherocytosis
- Elliptocytosis
- Sickle cell
- Hypochromic
- Microcytic
Classification of anemias: Characteristics of hereditary anemia?
- RBC membrane and cytoskeleton deficiency
- Hemoglobinopathies: sickle cell, thalassemia
- Enzyme deficiencies
Classification of anemias: What types of anemia are categorized as deficient erythropoiesis?
- “Stem cell” defect
- Maturation defect
Classification of anemias: RBCs of “stem cell” defect anemia?
- Normocytic, normochromic
- Normocytic, mildly macrocytic
Classification of anemias: Examples of “stem cell” defect anemias (list 2)
- Aplastic anemia
- Pure red cell aplasia
Classification of anemias: RBCs of maturation defect anemia?
- Megaloblastic
- Anisocytic
- Hypochromic
- Poikilocytic
Classification of anemias: Characteristic of maturation defect anemia?
- Pernicious anemia
- Folate deficiency
- Iron deficiency
Mechanisms of anemia: Examples of blood loss that cause anemia?
- Acute: trauma
- Chronic: GI, malignancy, menstrual bleeding
What are schistocytes?
fragmented RBCs
Mechanisms of anemia: Examples of external factors that could increase destruction of RBCs?
- Ab mediated transfusion Rx
- Erythroblastosis fetalis
- Autoimmune
- Trauma to RBC
- Infection
- Sequestration in spleen.
Mechanisms of anemia: Examples of hereditary internal factors that could increase destruction of RBCs?
- Cytoskeleton defect (hereditary spherocytosis)
- Structurally abnormal Hgb (sickle cell, thalassemia)
- Enzyme deficiency (G6PD)
Mechanisms of anemia: Other than external and hereditary internal factors, what are other sources of increased destruction of RBCs?
- Acquired defect
- Paroxysmal nocturnal hemoglobinuria (= an acquired, life-threatening disease in which hemolysis, thrombosis, and anemia occur)
Describe paroxysmal nocturnal hemoglobinuria
- Etiology?
- characterized by which 3 things?
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired, life-threatening disease of the blood. The disease is characterized by destruction of red blood cells (hemolytic anemia), blood clots (thrombosis), and impaired bone marrow function (not making enough of the three blood components).
Paroxysmal nocturnal hemoglobinuria: Red cells are more prone to lysis by what mechanism?
Complement
Paroxysmal nocturnal hemoglobinuria: Condition results in what venous condition?
venous thrombosis
Paroxysmal nocturnal hemoglobinuria: CLinical signs?
- Episodic hemoglobinuria, 1st morning urine.
Paroxysmal nocturnal hemoglobinuria: How does venous thrombosis manifest?
- Hepatic
- Portal
- Mesenteric
- Cerebral veins. (Harry potter makes cake)
Mechanisms of anemia: How can decreased production of RBCs occur?
- Disturbance of stem cells
- Defective heme production
- Defective DNA production
- Destruction of bone marrow
Mechanisms of anemia: What is a condition that can cause a disturbance of stem cells?
aplastic anemia
Mechanisms of anemia: What are conditions that can cause a defective heme production? (list 2 ex’s)
- IDA
- thalassemia
Mechanisms of anemia: What is a condition that would lead to defective DNA production?
- Vitamin B12 deficiencies
- Folic acid deficiencies
Mechanisms of anemia: What is a condition that could lead to a destruction of bone marrow?
metastatic tumor
Sites of removal of RBC from circulation: Extravascular site(s)?
- Phagocytic systems of spleen and liver
Sites of removal of RBC from circulation: Complications?
- Hemosiderin deposition in organs
- Jaundice and gallstones
Sites of removal of RBC from circulation: Why could jaundice and gallstones occur as a complication?
Results from elevated unconjugated bilirubin associated with hemolysis
Sites of removal of RBC from circulation: Intravascular source?
- Destruction of RBC in the vessel
Sites of removal of RBC from circulation: Intravascular complications?
- Acute tubular necrosis
- Jaundice and gallstones –> can occur due to increased unconjugated bilirubin
Why could acute tubular necrosis occur as a complication of intravascular removal of RBCs?
Due to hemoglobinemia
Lab findings in hemolysis of RBCs: Hgb/Hct?
Hgb decreased, Hct decreased
Lab findings in hemolysis of RBCs: Haptoglobin?
- Initially: increase
- Over time (net): Decreased
Lab findings in hemolysis of RBCs: Wy does haptoglobin decrease over time?
Because it keeps binding to that free heme circulating.
Lab findings in hemolysis of RBCs: LDH?
Increased lactate dehydrogenase (LDH) (it’s a liver enzyme and its inside RBCs)
-and increased unconjugated bilirubin
Lab findings in hemolysis of RBCs: K+?
Increased potassium (since cells are breaking down and releasing potassium)
What conditions might you see spherocytes with?
- Hereditary spherocytosis
- Immune hemolysis
What conditions might you see schistocytes with?
- TTP
- HUS
What conditions might you see target cells with?
hemaglobinopathies
What conditions might you see teardrops with?
Myelofibrosis
What conditions might you see sickle cells with?
- Sickle cell
- Thalassemia minor with sickle cell
What conditions might you see Rouleaux with?
Rouleaux= “stacking of coins”–> - due to Increased globins or decreased albumin (multiple myeloma)
Clinical presentation of anemia?
- Pale skin
- Fatigue
- Shortness of breath
- Chest pain
- Syncope and dizziness
Lab values associated with an initial anemia assessment?
- MCV
- Reticulocyte count
What does an increased reticulocyte count imply?
- Bone marrow intact and able to produce RBC
- Acute blood loss or hemolysis
What does a decreased reticulocyte count imply?
- Primary bone marrow disorder
OR - Deficiency in building blocks (iron, B12, folate)
MCV values: What are the categories of MCV?
- Microcytic
- Normocytic
- Macrocytic
MCV values: Microcytic?
Normocytic? Macrocytic?
Micro- < 80 fl
Normo- 80-100 fl
Macro- >100 fl
What are the microcytic anemias?
- Iron Deficiency
- Thalassemia
- Late stage anemia of chronic disease
Iron deficiency anemia: MCC in US? (list MC cause of blood loss in the US vs outside the US)
- GI blood loss or menstrual blood loss
- -Outside of US- due to poor nutrition
Iron deficiency anemia: Additional causes of IDA?
- Malabsorption
- Increased demand (pregnancy)
IDA labs: What would you look at?
- Ferritin
- Serum iron
- Iron stores in bone marrow
- TIBC
- RDW
- Transferrin saturation
IDA Labs: Results
- Decreased ferritin (less than 30 is diagnostic of IDA))**
- Decreased serum Iron
- Decreased iron stores in bone marrow
- Decreased transferrin saturation(ratio of serum iron to TIBC
- Increased TIBC
- Increased RDW