Bone Marrow Disorders Flashcards
Pure red cell aplasia (PRCA)
Severe reduction in the number of reticulocytes in the peripheral blood and the virtual absence of erythroid absence.
The bone marrow ceases to produce RBC’s
PRCA causes
Autoimmune disease Thymoma Viral infxns Large granular lymphocyte leukemia Idiopathic congenital
PRCA dx
Very low retic count and anemia when the following are present:
- normocytic, normochromic anemia
- Absolute retic < 10,000
- Normal WBC and platelet count
- Normal marrow, except no erythroid precursors
PRCA Tx
Red cell transfusions
Removal of cause
Usually relapses
Myelophthisic Anemia (MA)
Severe anemia caused by the displacement of hematopoetic bone marrow into peripheral blood.
MA Causes
Chronic myeloproliferative disease
Leukemia
Lymphoma
Metastatic carcinoma lymphopma
MA Dx
Tear drop RBC’s
Immature granulocytes
Treat underlying cause/cancer
Myelodysplastic Syndrome (MDS)
Heterogenous group of stem cell disorders characterized by dysplastic and ineffective blood cell production.
Dysplasia of one or more cell lines
AKA pre-leukemia
MDS patients have a reduction in which blood cells?
Normal RBC’s
Platelets
Granulocytes
MDS leads to?
Anemia
Bleeding
Increased risk for infxn
Who is affected by MDS?
Elderly (60 - 80)
Double the incidence of AML
Transformation of MDS to acute leukemia occurs in ___% of patients.
40%
Manifestations of MDS
Anemia (macro, normo) Oval macrocytes Neutropenia Thrombocytopenia Dysplastic features
Blasts account for ___ than 20% in MDS.
Less than 20%. If greater, consider Acute Myeloid Leukemia
MDS Tx
Bone marrow transplant. It is risky the older you get.
Aplastic anemia (AA)
Severe anemia characterized by pancytopenia accompanied by hypocellular bone marow
Aplastic causes
Direct stem cell injury (meds)
Autoimmune
SLE (rare)
How many cases of aplastic anemia are idiopathic?
70%
AA symptoms
Pallor Mucousal bleeding ecchymoses infxns gingivitis
AA lab findings
Pancytopenia normo, normo anemia Low retic Empty fatty bone marrow Few hematopoetic cells
AA Tx
Bone marrow transplant
Immunosuppression
Transfusions if needed
Withdraw etiological agent if found
Fanconi Anemia (FA)
Genetic disease, 1 in 350,000 births.
Bone marrow failure by 40
Development of AML
Usually diagnoses by 8 yrs
Is FA the most common cause of inherited bone marrow failure?
Yes.
FA Dx
Chromosome fragility test (MMC, DEB)
FA Tx
Transfusions
Androgens
Growth factors
Bone marrow transplant
Allogenic
Comes from another person
30 - 50% will die
Syngenic
Comes from an identical twin
Autologous
Comes from the patient
<5% will die
MUD
Matched unrelated donors
MRD
Matched related donors
25% chance of being a match
CD34+
Identification for stem cells
