Hematopoietic and Lymphoid Flashcards
What is the formation and development of blood cells
Hematopoiesis
Hematopoiesis occurs in the marrow of long bones: tibia, femur in children or aduts
Children
Hematopoiesis occus in the pelvis, cranium, vertebral bodies, sternum, ribs in children or adults
Adults
What is extramedullary hematopoiesis
Formation of blood cells outside of the medullary cavity (spleen, liver, lymph nodes, thymus)
A RBC undergoing hemolysis will release what
Hemoglobin (Hb)
Erythropoiesis occurs where
In red bone marrow
What is a Reticulocyte
Immature RBC (1% of circulating RBCs)
What is Polycythemia
Inc. RBCs
What is anemia
Dec. RBCs
What is the reason for a dec. RBC production anemia
Marrow Failure
How can the kidneys affect blood cell production
Inc. erythropoietin (Inc. erythropoiesis up to 8x)
What are the common S/S of anemia
Pallor, fatigue, weakness/lassitude
Rate of onset and mechanism have a direct impact on what with anemia
Severity
What does hypoxia cause the release of
Erythropoietin (EPO)
What is hemodilution
Inc. in plasma
What is reticulocytosis
Inc. in reticulocytes
Accelerated RBC destruction, hemolysis and dec. RBC life span refer to what type of anemia
Hemolytic
Hemolytic Anemia: What type of defects are hereditary: abnormal RBC membranes, enzyme deficiency, disordered Hb synthesis
Intracorpuscular
Hemolytic Anemia: What type of defects are Acquired: antibodies, RBC trauma, infxns. (malaria)
Extracorpuscular
What type of hemolysis is inside circulation, physical or biochemical damage, and releases Hb
Intravascular hemolysis
What type of hemolysis occurs inside the spleen and liver, RBC damage, antibody opsonization, splenomegaly, jaundice AND is MC
Extravascular hemolysis
What is a Poikilocyte
Abnormally shaped RBCs (general term)
What is a Shistocyte
Irregularly shaped RBCs, from hemolysis
What is a Dacrocyte
Teardrop-shaped RBC
Which Hemolytic Anemia:
Abnorm. RBC membrane
Fragile spherocytes removed by spleen, mod. anemia
Poss aplastic crisis parovirus B19 (5ths disease)
TX: Splenectomy
Hereditary Spherocytosis
Why are sites of blood stasis particularly bad in an individual with Sickle Cell anemia
The dec. O2 leads to sickling
What type of mutation is Sickle cell
B-globin, autosomal recessive
Heterozygous Sickle cell will result in what
Sickle cell trait = carriers, but not affected 8% Af. americans
Homozygous Sickle cell will result in what
Sickle cell anemia/disease, 1/600 Af. Americans
Which Hemolytic Anemia:
Thrombosis, fever, malaise, chronic low-level pain
Splenomegaly/infarction, gallstones, priapism
Stunted growth, osseous distortion
Sickle Cell Anemia
Sickle cell related, What condition is from lung infections or PE produces pulmonary stasis leading to dec. O2 leading to thrombosis
Acute chest syndrome
Sickle cell related, What condition results from a cerebrovascular obstruction
Stroke
Sickle cell related, What condition can affect the heart, kidneys, and liver
Hypoxia-induced fatty changes
T/F: With Homozygous Sickle Cell 60% survive beyond the 5th decade
FALSE, 50% survive beyond the 5th decade
In sickle cell anemia, where is the MC place for and infarction
Bone marrow
What distinct sign can possibly be seen on xray in the vertebra
H-shaped vertebra aka Lincoln Log Vertebrae (10%)
Which Hemolytic Anemia:
Abnormal hemoglobin, mutated alpha or beta globin genes, autosomal recessive, seen most in areas of endemic malaria
Thalassemia
What does the mutation in thalassemia cause
excess of the opposite globin chain and damages RBCs (hemolysis) as well as erythroblasts
If you have to have beta thalassemia, which do you want to have
Minor, 1 allele, subtle/very mild if any symptoms
Severe hemolysis and anemia, extramedullary hematopoiesis, splenomegaly, stunted growth, bone marrow expansion/distortion describes what condition
Beta-thalassemia major, 2 alleles
T/F: Tx. may not be necessary for beta-thalassemia minor
True!
What is the Tx for beta-thalassemia major
Repeated transfusions and iron chelation OR Bone marrow transplant may cure
Even with Tx, once a person is in their 20s, what will most likely be their downfall
Lethal Dilated Cardiomyopathy
What 2 xray signs can be seen in beta-thalassemia major
“hair on end” and “lace-like trabeculation”
What distinct facial features may be seen in beta-thalassemia major
Chipmunk face
Which Hemolytic Anemia:
Abnormal Hb, Slight dec. O2 capacity, ineffective erythropoiesis, highly variable, determined by # of altered genes
Alpha-Thalassemia
G6PD is needed to make Glutathione (GSH), which is a powerful
Antioxidant, protects against oxidative stress
G6PD Deficiency is MC in
Males
What is significant about G6PD deficiency
Asymptomatic until exposed to oxidative stress (infxns. MC)
G6PD Bite cells vs Heinz bodies
Bite:splenic macrophages
Heinz: oxidized Hb, clump together
What is the Tx for G6PD deficiency
Depends on severity, but Id/terminate oxidative stress, blood transfusion, partial splenectomy
Which Hemolytic Anemia:
Dark urine upon waking
Acquired PIGA gene mutation
X chromosome, but can still affect females
Paroxysmal Nocturnal Hemoglobinuria
T/F: PNH is the MC mild/ chronic low-level anemia
True
People with PNH are at an inc. risk for what
venous thrombosis
What is the tx for PNH
antibodies that inhibit the MAC, or marrow transplant (curative)
How are Immunohemolytic anemias Dx.
Via direct Coombs antiglobulin test
Spontaneous and idiopathic describes what kind of stimuli in immunohemolytic anemias
Endogenous
Toxic exposure or ADRs describes what kind of stimuli in immunohemolytic anemias
Exogenous
What type of Immunohemolytic Anemia:
IgG opsonization, most are primary = idiopathic, 25% develop secondary
Warm antibody type
What type of Immunohemolytic Anemia:
Periphery, IgM opsonization prim or sec to infxn or B cells lymphoma
Cold antibody type
Which Hemolytic Anemia:
Repetitive physical trauma to RBCs, prosthetic heart valves (blender effect), narrowing of vessels, RBCs become shistocytes, MC asymptomatic
Traumatic Hemolysis
Plasmodium falciparum, protozoan, female anopheles mosquito, leads to what condition
Malaria
Where is malaria MC
Asia and Africa