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Hematology Lecture 15: LMU
Terms in this set (42)
What are the Normo-normo anemias?
- Aplastic Anemia (3 types)
- Pure Red Cell Aplasia (3 types)
- Congenital Dyserythropoietic Anemia
- Acute Blood Loss Anemia
- Renal Disease Anemia
- Endocrine Disease Anemia
- Myelophthisic Anemia (Leukoerythroblastosis)
What are the results of bone marrow failure?
- all blood cell production stops
- severe reduction or cessation of blood cell production and/or increased blood cell destruction in the bone marrow
- pancytopenia in BOTH the peripheral blood and the bone marrow
What is the clinical picture for bone marrow failure?
- easy bruising, bleeding issues due to low platelets
- RBC life span decreased
What is the most common & most severe of the bone marrow failure syndromes?
What 3 things characterize Aplastic Anemia?
- bone marrow failure
- CD34 positive stem cells can be 10x lower
- pancytopenia (peripheral blood)
- high serum iron levels
- no reticulocytosis
low retic count
Normal & Abnormal Hypoplastic BM Sections:
What are the 3 forms of Aplastic Anemia (AA)?
- Congenital (Inherited) AA
- Acquired AA
- Idiopathic AA
What is the congenital form of AA?
- chemicals, drugs, radiation, heavy metals, virus, infection, etc.
- 90% are caused by drugs
- viruses such as Hep A, Hep B, Epstein-Barr, CMV, & B19 parvovirus
What antibiotic is implemented in most often implicated in the development of AA?
What type accounts for 50% of all cases of AA?
What are the symptoms of AA?
- pallor, fatigue, weakness
- bacterial infections due to neutropenia
What lab results can you expect for AA?
- normal MCV, MCH, & MCHC
- prolonged bleeding time
low to absent retic
- relative lymphocytosis w/ neutropenia
- high serum iron
- transferrin is saturated
- BM - hypocellular, increase in yellow marrow
What is the treatment for AA?
- blood products for immediate treatment
- bone marrow transplant w/ HLA identical sibling
- immunosuppressive therapy w/o HLA identical sibling
What is Fanconi's Anemia?
- congenital AA
- progressive pancytopenia
- autosomal recessive
What are the physical abnormalities in Fanconi's Anemia?
- encephaly (abnormally small brain)
- short stature
- crossed eyes
- malformation of the kidneys
- mental retardation
- thumb malformations
What is the treatment for Fanconi's Anemia?
bone marrow transplant
Fanconi's Anemia Lab Findings:
- 5 to 10 years of age
- pancytopenia - PB & BM
- high HgB F
- defect in rejoining of breaks in the DNA chains
What is Pure Red Cell Aplasia (PRCA)?
- rare disorder of erythroplasia in an otherwise healthy bone marrow
- severe anemia
- only erythroid precursor are affected
PRCA in children is known as:
What is the congenital form of PRCA?
Diamond-Blackfan Anemia Lab Findings:
- < 1 year of age
- marked decrease in erythroid precursors only - BM
- decrease in RBC - PB
- normal WBC & plt - PB
AA vs. PRCA:
- low numbers of both myeloid & erythroid precursors
- normal cellularity of myeloid cells, megakaryocytes
- erythroid hypoplasia
- remaining erythroids are immature
What is the etiology of PRCA?
- immunoglobin inhibitor of erythroid precursors
----- in-vivo incubation of patients own's serum and bone marrow cells: inhibition of erythroid growth
----- in-vitro incubation w/o patient's serum: patient's erythroid cells do grow
PRCA lab findings:
- severe normo-normo anemia (slightly macrocytic)
- idiopathic or immune mechanism
- decreased erythroid precursors in BM
- normal WBC & plt
- increased EPO
- decreased retic
- BM: lots of cellularity, marked decrease or absence of erythroid precursors
- more common
- secondary to other conditions
----- thyroid tumors
----- parvovirus B19 - rubriblasts become infected w/ virus and "die off" before maturing
- Diamond-Blackfan Anemia
- infants & young children
Congenital Dyserythropoietic Anemia (CDA):
- ineffective erythropoiesis
- secondary hemosiderosis
- giantism of bone marrow cells
- multinuclearity of bone marrow cells
What is Type I CDA?
- both sexes
- basophilic stippling
- cabot's rings
- multinucleation, incomplete nuclear segments, internuclear chromatin bridges, and unevenly dense nuclear chromatin, RBC precursors appear megaloblastic
What is Type II CDA?
- most common type
- same features as Type I but RBC precursors are not megaloblastic
What is Type III CDA?
- rare, inherited a autosomal dominant
- erythrocytes have megaloblastic features, with as many as 12 nuclei in some cells
Acute Blood Loss Anemia:
- trauma in which a significant amount of blood is lost within a short period of time
What are the symptoms of Acute Blood Loss Anemia?
- low blood pressure
- rapid pulse
A loss of 30-40% of the total blood volume leads to ________, _______, and/or loss of ___________.
- shortness of breath
Acute Blood Loss Anemia lab findings:
- H/H normal immediate after, due to vasoconstriction
- H/H drops within 3-4 hours
- Plts & WBCs increase with "left shift"
In Acute Blood Loss Anemia, the retic becomes elevated within ______, peaks at _______, and remains elevated until ____ returns to normal.
- 2-3 days
- 6-10 days
What is Anemia of Renal disease?
- normo-normo anemia due to failure of kidneys to produce EPO
- few macrocytes may be present in patients on dialysis, due to loss of folic acid
- platelet function is impaired due to toxic metabolites
- high BUN
- high creatinine
are often present
- treatment: recombinant human EPO injections
What is Anemia of Endocrine diseases?
- many hormones are involved in the regulation of erythropoiesis, and deficiencies of such hormones lead to anemia
Leukoerythroblastosis (Myelophthisic Anemia):
- normo-normo anemia
- infiltration of abnormal cells into the bone marrow and subsequent destruction and replacement of normal hematopoietic cells
- metastatic solid tumor cells such as lung, breast, and prostate are often implicated
- associated with "space-occupying" disorders of the BM
----- carcinoma is invading the BM and is pushing out NRBC and immature granulocytes
Leukoerythroblastosis lab findings in PB:
- normal to low plt w/ occasional bizarre shape of plts
unusual presence of NRBCs
----- NRBCs only appear in PB: severe anemia
----- HgB may be 11 g/dL / NRBCs present
low WBC w/ immature granulocytes (meta & myelos)
- "left shift"
- enzyme defects in heme synthesis
- may be micro-hypo or normo-normo
- inherited porphyrias: genetic problem, rare
- acquired porphyrias: lead poisoning, toxic chemicals, drugs
Summary Sheets to Study:
THIS SET IS OFTEN IN FOLDERS WITH...
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