Anemia Flashcards
ANEMIA can be a result of:
An absolute failure of the bone marrow to replace those erythrocytes that are normally destroyed after 120 days
Premature destruction (such as seen in hemolysis)
Types of anemia
ANEMIA OF: BONE MARROW FAILURE SYSTEMIC DISORDERS ABNORMAL NUCLEAR DEVELOPMENT ABNORMAL IRON METABOLISM ABNORMAL GLOBIN DEVELOPMENT INCREASED ERYTHROCYTE DESTRUCTION BLOOD LOSS
ANEMIA OF BONE MARROW FAILURE
APLASTIC ANEMIA
There is peripheral blood pancytopenia (that is, a decrease in ALL formed elements of the blood)
Bone marrow is severely hypolastic or aplastic
APLASTIC ANEMIA
underdevelopment or incomplete development of a tissue or organ
HYPOPLASIA
failure of a tissue or organ to develop
APLASIA
The name can be misleading as it implies that the anemia is the main problem; however, the most serious complications arise from _____ brought about by ____ and ___ from ______
severe infections - leukopenia
bleeding from thrombocytopenia
DIAGNOSTIC CRITERIA FOR SEVERE APLASTIC ANEMIA
BONE MARROW
Cellularity__ of normal or __ of normal cellularity with __ hematopoietic cells
Plus any two of the following:
Granulocytes
Platelets
Anemia with
-
-
Seen maximally at the age of ____
There are no immature myeloid cells in the peripheral blood
There is an aplastic or hypoplastic marrow (that is one replaced by fat)
Lack of splenomegaly
-
6-50 years
2-5X more frequent in the Far East (i.e. Japan, China, Korea) than North America or Europe
aplastic anemia
Rare, inherited form of aplastic anemia
Autosomal recessive
FANCONI ANEMIA
Congenital Aplastic Anemia
FANCONI ANEMIA
Congenital Aplastic Anemia
Seen coupled with:
8
Microcephaly Brown skin pigmentation Short stature Malformation of the thumb Internal strabismus (cross eyes) Renal malformations Genital hypoplasia Mental retardation
A rare condition that involves either an idiopathic or an immune mechanism
Some patients may have an Ig inhibitor of erythroid precursors or erythropoietin inhibitor
May be due to a benign disease, drugs, chemicals, infections, or hemolytic anemia-aplastic crisis
ACQUIRED PURE RED CELL APLASIA
Some patients may have an Ig inhibitor of erythroid precursors or erythropoietin inhibitor
ACQUIRED PURE RED CELL APLASIA
May be due to a benign disease, drugs, chemicals, infections, or hemolytic anemia-aplastic crisis
ACQUIRED PURE RED CELL APLASIA
- Congenital Hypoplastic anemia or PRCA
- A rare congenital disorder defined as a normochromic-normocytic anemia with normal leukocyte and platelet count and a marked decrease in marrow normoblasts
- First described in 1938
- Etiology is unknown, however one in vitro study shows the the colony-forming units-erythroid (CFU-E) were insensitive to erythropoietin stimulation
DIAMOND BLACKFAN ANEMIA
Congenital Pure Red Cell Aplasia
DIAMOND BLACKFAN ANEMIA
Congenital Pure Red Cell Aplasia
- _______ or PRCA
- A rare congenital disorder defined as a __________ with normal ____ and _____ and a marked decrease in marrow ______
- First described in ____
- Etiology is unknown, however one in vitro study shows the the colony-forming units-erythroid (CFU-E) were insensitive to _______
Congenital Hypoplastic anemia
normochromic-normocytic anemia
leukocyte and platelet count
normoblasts
1938
erythropoietin stimulation
A common finding in 94% of patients with carcinoma
The degree of anemia correlates with the degree of ____ burden
Sometimes used interchangeably with _______________
MYELOPHTHISIC ANEMIA
tumor
leukoerythroblastic reaction
NOTE:
The _________ is the presence of circulating nucleated rbc (NRBC) and immature leukocytes in the peripheral blood; THIS REACTION IS NOT ASSOCIATED WITH ANEMIA.
MYELOPHTHISIC ANEMIA
LEUKOERYTHROBLASTIC REACTION
Aplastic anemias which have a number of identified causative factors and agents.
Radiation
Seen in long term, low dose irradiation where radiation may damage the stem cells
High dose radiation associated with acute exposure (radiotherapy, radioactive isotope administration, or work in unsafe power plants)
Immune Mechanisms
Caused either directly by lymphocytes or by some humoral factors
SECONDARY APLASTIC ANEMIAS
SECONDARY APLASTIC ANEMIAS
Drugs and Chemicals
________is the classic drug associated with marrow aplasia
Others include 5
Chemicals include: 3
Chloramphenicol
benzene and benzene derivatives, hydantoins, sulfonamides, and gold preparations
Chlordane,
Chlorophenothane (DDT) and
gamma benzene hexachloride (Lindane)
Radiation
Seen in long term, low dose irradiation where radiation may damage the stem cells
High dose radiation associated with acute exposure (radiotherapy, radioactive isotope administration, or work in unsafe power plants)
–
Caused either directly by lymphocytes or by some humoral factors
SECONDARY APLASTIC ANEMIAS
Immune Mechanisms
Laboratory Findings Peripheral Blood: - - - - - -
Bone Marrow: - - - - - -
Pancytopenia
Normocytic-Normochromic type of anemia with slight macrocytosis
Normal red cell morphology
Reticulocyte Count is decreased
Reticulocyte Production Index is severely decreased
NO IMMATURE CELLS in the peripheral blood
Hypoplastic or aplastic Increased fat replacement Decreased hematopoietic cells Principal cells present are lymphocytes and plasma cells No increased number of immature cells Patchy areas of cellularity
used to evaluate patients with suspected PNH (______) or suspected congential dyserythropoietic anemia
Diagnosis of PNH shows that the suspected patient’s red cells have a high sensitivity to complement mediated hemolysis.
Ham Acid Serum Test
Paroxysmal Noctural Hemoglobinuria
Positive test result shows lysis of Red cells in acidified serum samples with patients cell (not with normal cells).
Ham Acid Serum Test
Lab Findings
Special Hematology Tests:
4
Chemistry:
3
Hemoglobin F (HbF) – elevated
Kleihauer-Betke Acid elution test for HbF– uneven distribution in red cells
Leukocyte alkaline phosphatase (LAP) – increased
Ham acid-serum test (confirmatory test for PNH) – maybe positive
Serum iron – Elevated
Plasma Iron Clearance – Delayed
Cytogenetics:
Marrow chromosomes – normal (when abnormalities are found, think of MYELODYSPLASTIC SYNDROME)
Treatment
Bone marrow transplantation
Patients should receive prior treatment with _____ to reduce the risk of rejection
Blood transfusions
Immunosuppressive therapy (antithymocyte globulin [ATG] or antilymphocyte globulin [ALG])
Androgens (that stimulate erythropoiesis and to a lesser degree, granulopoiesis and platelets)
cyclophosphamide
Failure of sustained marrow engraftment (i.e. rejection)
Development of graft versus host disease (GVHD)
Defined as an immune reaction trigerred by an incompatibility between the donor and recipient HLA antigens
Is mediated by donor lymphocytes acting against the host tissues
Appears anywhere between ___ after grafting which attacks the skin, liver, and the gut
Responsible for __ of mortality
Day 10 to 70
12%
Transplant Complications
Can be prevented by:
___ (though 25-30% of patients receiving this drug still develop GVHD)
____, which is a newer drug effective in the treatment of GVHD, particularly after kidney transplantation
Treated by ____, sometimes in combination with azathioprine
Methotrexate
Cyclosporin A (CyA)
prednisone
Prognosis
Patients who undergo bone marrow transplantation have a more favorable prognosis and survival rate than those from whom no donor can be found, especially after 6 months after diagnosis.
Patients who undergo bone marrow transplantation have a more favorable prognosis and survival rate than those from whom no donor can be found, especially after 6 months after diagnosis.
Anemias of Systemic Disorders
REMEMBER:
____________!
Hormones play a BIG ROLE in hematopoiesis
ANEMIA OF CHRONIC RENAL FAILURE
A ___ that can be severe, almost invariably occurs in patients with chronic renal failure (CRF)
Related to the etiology of renal disease:
-Failure of renal excretory function with resultatnt -accumulation of waste products
Failure of renal production and release of erythropoietin
Uremia is always often present
hypoproliferative anemia
Uremia is always often present
ANEMIA OF CHRONIC RENAL FAILURE
A hypoproliferative anemia that can be severe, almost invariably occurs in patients with chronic renal failure (CRF)
ANEMIA OF CHRONIC RENAL FAILURE
Related to the body’s low oxygenation
ANEMIA OF ENDOCRINE DISORDERS
HYPOTHYROIDISM
Anemia occurs because the pituitary gland influences the functions of the thyroid (through TSH), gonads Through FSH and LH), and adrenals (through ACTH)
The anemia is caused by ____ and a resultant ___ in stimulation of ______ production
Anemia is _____, with N/N red cell morphology
_____ stimulates the release of erythropoietin as well as ____ and ____
HYPOPITUITARISM
reduced metabolic demands
decrease
erythropoietin production
mild to moderate
Vasopressin
growth hormone
prolactin
Most often related to iron deficiency or folate deficiency
Anemia of Pregnancy
Anemia occurs due to deranged cortisol secretion that have multiple effects on blood cells, most markedly with circulating eosinophils and lymphocytes.
_____ – hypocortisolism
_____ – hypercortisolism
ANEMIA OF ENDOCRINE DISORDERS
ADRENAL ABNORMALITIES
Addison’s disease
Cushing’s disease
Is characterized by retarded growth and secondary sexual development
REMEMBER: ____
ANEMIA OF ENDOCRINE DISORDERS
HYPOGONADISM
Testosterone is an androgen
