Hematopathology Part I

Front 1

Hematocrit

Back 1

Volume occupied by RBC's.

33-49% is normal.

Front 2

Hemoglobin

Back 2

2 alpha and 2 beta chains and an iron heme

Front 3

Anemia

Back 3

Reduction of the oxygen transportation of the blood.

RBC #
Bad Hemoglobin

Front 4

Acute Blood Loss Anemia

Back 4

Hypovolemic shock is immediate concern.

Extent surfaces 2-3 days later

Normocytic, and normochromic

Front 5

Chronic Blood Loss Anemia

Back 5

Rate of loss exceeds regeneration

Results in IRON DEFICIENCY anemia

Front 6

Hemolytic Anemias

Back 6

Destruction of RBC's, due to defect or external factors.

Products of hemolysis are retained (iron causes HEMOCHROMATOSIS) and can cause jaundice.

Front 7

Intravascular Hemolysis

Back 7

Abnormally small blood vessels (often due to obstruction) causes RBC destruction.

Front 8

Extravascular Hemolysis

Back 8

Entrapment of RBC's in the SPLEEN where phagocytes destroy them.

Causes SPLENOMEGALY

Front 9

Sickle Cell Anemia (General)

Back 9

Caused by POINT MUTATION in BETA globin gene.

8% Af. Americans are heterozygotes.

Some MALARIA protection

Front 10

Factors that Affect Sickling

Back 10

Low Oxygen tension

Hemoglobin concentration in the cell

Length of time cells are exposed to low oxygen tension

Front 11

Sickle Cell Anemia Clinical Features

Back 11

RBC's become sickled, which causes PAIN due to ISCHEMIA.

Sickle cells are destroyed in the SPLEEN

"Hair on end" and expansion of bone marrow.

Front 12

Acute Chest Syndrome

Back 12

Due to a fat embolus from necrotic marrow or secondary pulmonary infection.

Front 13

Thalassemia

Back 13

Caused by decrease production of Alpha or Beta globin chains.

Offers some malaria protection.

Front 14

Beta Thalassemia (Single Gene)

Back 14

EXCESS ALPHA GLOBIN CHAINS cause precipitation and membrane damage.

Minor = Heterozygotes
Major = Homozygotes

Treated with lots of transfusions which leads to IRON OVERLOAD

Front 15

Alpha Thalassemia (Two Genes)

Back 15

EXCESS BETA CHAINS, but anemia is usually less severe.

Loss of Single Allele=Silent Carrier
Loss of Two Alleles=Asymptomatic Thalasemia
Loss of Three Alleles=HbH Disease with severe Anemia
Loss of Four Alleles=Fetal Death

Front 16

Glucose-6-Phosphate Dehydrogenase Deficiency

Back 16

Can't convert glutathione to reduced form (GSH which inactivates damaging oxidants)

Hydrogen Peroxide builds up and globin chains are damaged.

Inclusions called HEINZ bodies are formed, which can be attacked to become "BITE CELLS)

Front 17

Glucose-6-Phosphate Dehydrogenase Deficiency Clinical Findings

Back 17

Symptoms present only AFTER infections, certain drugs, or toxins

Avoid sulfonamides, aspirin, and antimalarials

Front 18

Traumatically Induced Hemolytic Anemias

Back 18

More severe with ARTIFICIAL VALVES

Microangiopathic Anemia causes RBC damage

Front 19

Malaria

Back 19

PLASMODIUM FALCIPARUM

Infection causes RBC destruction

Drug resistant strains of falciparum have emerged.

Front 20

Anemias of Diminshed Erythropoiesis

Back 20

Usually caused by inadequate supply of substances needed to create RBC's

Front 21

Iron Deficiency Anemia

Back 21

Caused by:
Chronic Blood Loss
Increased demand (pregnancy/infancy)
Malabsorption

Spooning of Nails

Glossitis

Front 22

Megaloblastic Anemia

Back 22

Nutritional deficiency of FOLATE or impaired B12 absorption

Delayed mitotic division with normal maturation causes ENLARGEMENT

Front 23

Folic Acid Deficiency

Back 23

Usually poor diet or pregnancy

Folate is easily destroyed during cooking

Absorption can also be the problem

Sore tongue, Glossitis, and Cheilosis

Front 24

Vitamin b12 Deficiency (Pernicious Anemia)

Back 24

Usually lack of INTRINSIC FACTOR from PARIETAL cells.

Causes demyelinating disease (neurological problems)

Injections solve the problem, but not oral medications!

Front 25

Causes of Pernicious Anemia

Back 25

Autoimmunity against PARIETAL CELLS

Gastrectomy or rescetion of ileum

Diseases of distal Ileum

Gastric atrophy in the elderly

Front 26

Aplastic Anemia

Back 26

Suppression of multipotent myeloid stem cells resulting in pancytopenia.

Causes:
Idiopathic
Radiation
Drugs
Infection

Lack of bone marrow (can be improved with transplant)

Front 27

Myelophthisic Anemia

Back 27

CROWDING of marrow (Gaucher's disease)

Most often associated with metastatic cancer.

Front 28

Bleeding Time (BT)

Back 28

Time for a small skin puncture to stop bleeding.

Assesses platelet formation

2-9 minutes is normal.

Front 29

Prothrombin Time (PT)

Back 29

Tests integrity of EXTRINSIC and common clotting pathways.

International Normalized Ration (INR) standardizes the values.

Front 30

Partial Thromboplastin Time (PTT)

Back 30

Tests integrity of INTRINSIC and common pathway

Front 31

Thrombocytopenia

Back 31

Less than 100,000 platelets/µL.

Causes:
1. Decrease in platelet production due to marrow failure/injury or tumor infiltration.
2. Accelerated destruction (immunologically mediated, drug-induced, AIDS, etc.).

Prolonged BT

Front 32

Idiopathic Thrombocytopenic Purpura (ITP)

Back 32

Autoimmune disease (anti-platelet immunoglobulins).

Front 33

Thrombotic Thrombocytopenic Purpura (TTP)

Back 33

Widespread formation of hyaline thrombi in the microcirculation

Front 34

Coagulation Disorders

Back 34

von Willebrand disease

Hemophilias

Vitamin K Problems

Front 35

von Willebrand Disease

Back 35

Inherited bleeding disorder

Prolonged bleeding time (but normal platelet count), prolonged PTT.

Front 36

Hemophilia A (Classic Hemophilia)

Back 36

Caused by reduced amount or reduced activity of FACTOR VIII.

X-linked recessive

Spontaneous hemorrhages in the joint space (hemarthrosis) which may be crippling.

Prolonged PTT.

Front 37

Hemophilia B (Christmas disease)

Back 37

Clinically INDISTINGUISHABLE from hemophilia A.

X-linked recessive inheritance.

Deficiency of FACTOR IX

Prolonged PTT