Flashcards in 3.24.14* Goorha - Bone Marrow Failure Deck (24):
Symptoms of anemia including
difficulty breathing, chest pain and fatigue.
Symptoms of leukopenia/neutropenia include
fever, infection and mouth sores.
causes of pancytopenia by increased destruction
Causes of pancytopenia by decreased production
causes of hypercellular marrow
bone marrow infiltration
hematologic malignancies (leukemia, myeloma, lymphoma)
B12 or folate deficiency
causes of hypercellular marrow
a. congenital (fanconi's anemia)
b. aquired (idiopathic, myelodysplastic syndrome, drugs/chemicals, radiation, viruses)
characterized by peripheral pancytopenia and hypocellular bone marrow.
- This may be due to quantitative or qualitative damage to the pluripotential stem cell
- or the result of a defective bone marrow microenvironment
- or from cellular or humoral immunosuppression of hematopoiesis.
What is a primary mechanism of idiopathic aplastic anemia?
a. Infiltration of bone marrow by abnormal protein
b. Immune mediated destruction of hematopoietic stem cells
c. Immune mediated destruction of peripheral blood cells
d. Vitamin deficiency
the disorder usually becomes symptomatic ~5 years of age and is associated with progressive bone marrow hypoplasia. Congenital defects such as skin hyperpigmentation and small stature are also seen in affected individuals.
Infections that can lead to aplastic anemia
viral and bacterial infections such as infectious mononucleosis, infectious hepatitis, parvovirus and cytomegalovirus infections, and miliary tuberculosis occasionally lead to aplastic anemia
Lab findings in aplastic anemia
a. Severe pancytopenia with relative lymphocytosis (lymphocytes live a long time)
b. Normochromic, normocytic RBCs (may be slightly macrocytic)
c. Mild to moderate anisocytosis and poikilocytosis
d. Decreased reticulocyte count
Hypocellular bone marrow with > 70% yellow marrow
What is/are treatment option(s) for aplastic anemia?
Immunosuppression (this suggests auto-immune destruction is present in idopathic cases)
Stem cell transplant
Pure red cell aplasia
is characterized by a selective decrease in erythroid precursor cells in the bone marrow. WBCs and platlets are unaffected
Causes of pure red cell aplasia
a. Transitory with viral or bacterial infections
b. Patients with hemolytic anemias may suddenly halt erythropoiesis
c. Patients with thymoma – T-cell mediated responses against bone marrow erythroblasts or erythropoietin are sometimes produced
Treatment is supportive care and immunosupression
What is the primary difference between myelodysplastic syndrome and aplastic anemia?
a. Presence or absence of pancytopenia
b. Presence or absence of impaired hematopoesis
c. Presence or absence of neoplastic cells in the bone marrow
d. Spleen size
a. primary, neoplastic stem cell disorders that tend to progress to acute leukemia (>20% blasts)
b. normocellular or hypercellular BM with dysplastic changes resulting ineffective erythropoiesis and or granulopoiesis and or megakaryopoiesis
c. increased level of apoptotic cell death in bone marrow cells
d. increase in blasts
What is seen in PBS or MS
dysplastic (abnormality in development) cells including nucleated RBCs, oval macrocytes, pseudo-Pelger-Huet PMNs (hyposegmented neutrophils) with hyperchromatin clumping, hypogranulated neutrophils, and giant bizarre platelets.
What is seen in the bone marrow of patient with myelodysplastic syndrome?
Normo- or hypo-cellular
What is the treatment for myelodysplastic syndrome?
transfusion if needed
(only trying to prevent progression to AML)
causes tumor cell apoptosis by inhibiting stromal cell support.
Used in myelodysplastic syndrome and multiple myeloma.
autologous v. allogeneic BMT
in general think autologous for lymphoma and multiple myeloma
allogeneic for leukemia and myelodysplastic syndrome, aplastic anemia, some lymphomas
18 year old African American male presented to Methodist Hospital with complaint of fatigue and ‘passing out’
On presentation, wbc = 0.8, hgb = 5.5, platelets = 40,000
No family history of blood problems
Exam was unremarkable
treatment for aplastic anemia
Anti-thymocyte globulin, steroids, and cyclosporine