Bone Marrow Disorders Flashcards
What is Pure Red Cell Aplasia (PRCA)?
a type of anemia affecting the precursors to red blood cells but not to white blood cells, or platelets.
What are Pure Red Cell Aplasia (PRCA) Characteristics?
a severe reduction in the number of reticulocytes in the peripheral blood and absence of erythroid precursors in the bone marrow
What are Pure Red Cell Aplasia (PRCA) Causes?
Autoimmune disease. Thymoma. Viral infections. Large granular lymphocyte leukemia. Idiopathic
Congenital - Diamond-Blackfan anemia.
How is Pure Red Cell Aplasia (PRCA) Diagnosis made?
normocytic normochromic anemia. absolute reticulocyte count <10,000/microL. normal WBC and platelet counts. normocellular bone marrow with normal myelopoiesis and megakaryocytopoiesis, but few if any erythroid precursors
What is Pure Red Cell Aplasia (PRCA) Treatment?
red cell transfusions for symptomatic anemia. cessation of possible offending drugs.Treatment of the underlying condition
What is Myelophthisic Anemia?
severe anemia due to the displacement of hemopoietic bone-marrow tissue into the peripheral blood, either by fibrosis, tumors or granulomas.
What are Myelophthisic Anemia Causes?
Chronic myeloproliferative disease (e.g. myelofibrosis).Leukemia/Lymphoma. Metastatic carcinoma or myeloma. linked to small-cell lung cancer, breast cancer and prostate cancer that metastasizes to the bone marrow
How is Myelophthisic Anemia Diagnosis made?
Blood smear: erythrocytes that contain nuclei or are tear drop-shaped (dacryocytes), or immature granulocyte precursor cells. Bone marrow biopsy shows replacement of the normal bone marrow by fibrosis, malignancy or other infiltrative process.
What are Myelophthisic Anemia Treatment options?
treatment of the underlying cause/cancer.
What is Myelodysplastic syndrome (myelodysplasia or MDS)?
hematopoietic stem cell disorders characterized by dysplastic and ineffective blood cell production.
What is the Morphologic hallmark of MDS?
Dysplasia of one or more cell lines
Why is MDS also know as “preleukemia”?
it has a variable risk of transformation to acute leukemia (occurs in up to 40% of patients)
What are possible systemic complications of MDS?
Anemia, Bleeding, Increased risk of infection
What is the epidemiology of MDS?
the elderly, with a median age at diagnosis of between 60-80 years.
What lab results are seen with MDS?
macro-, normocytic (occasionally microcytic) anemia. oval macrocytes on peripheral smear. Neutropenia. Thrombocytopenia. Differentiated from AML by the presence of less than 20% blasts.
Patients with MDS have a variable reduction in the production of ?
normal red blood cells, platelets, and mature granulocytes.
How do you treat MDS?
Consider transplant in the young, or investigational Rx. supportive (RBC transfusions, treat infections). Growth factors, erythropoietin effective in some patients
What are MDS Indicators of a good prognosis?
Younger age. Normal or moderately reduced neutrophil or platelet counts. Low blast counts in the bone marrow(<20%) and no blasts in the blood;
No Auer rods. Normal karyotypes without complex chromosome abnormalities
What is aplastic anemia?
characterized by peripheral pancytopenia and accompanied by a hypocellular bone marrow.
What can cause Stem cell failure?
1-Direct stem cell injury (Radiation, chemotherapy, toxins, meds). 2 – Autoimmune suppression of hematopoiesis – most common. 3 – Rarely SLE
What are Secondary Aplastic Anemia Causes?
Meds/ toxins (Chloramphenicol, carbamazapine, indomethacin, cimetidine, sulfas, acetazolamide, lithium, human growth hormone). Radiation
Viruses. Paroxysmal Nocturnal Hemoglobinurea. Malnutrition. Myelodysplastic syndromes.Thymoma
What are symptoms of aplastic anemia?
pancytopenia: pallor, mucosal bleeding, ecchymoses, or petechiae. infections. Hyperplastic gingivitis
What are expected lab results of aplastic anemia?
Pancytopenia, Normocytic-normochromic anemia, Low reticulocytes. Bone marrow biopsy-Empty fatty spaces, Few hematopoietic cells or Lymphocytes and plasma cells
What are Aplastic Anemia Treatment options?
Withdrawal of the etiologic agent. Blood and platelet transfusion. Allogeneic BMT. Immunosuppression (Cyclosporin + ATG, Corticosteroids, cyclophosphamide)
What is Fanconi Anemia (FA)?
genetic defect which causes the majority of FA patients to develop AML and bone marrow failure by age 40. Higher frequency in Ashkenazi Jews and Afrikaners in South Africa.
When is FA diagnosed?
as neonates/infants if abnormalities. Others may be diagnosed when hematological problems occur. age of onset of pancytopenia is 7. First develop macrocytosis, then thrombocytopenia, and eventually neutropenia.
What genetic tests help confirm FA diagnosis?
Chromosome fragility test: Mitomycin C (MMC) or diepoxybutane (DEB) added to lymphoctyes – increases the number of chromosome breaks and radial structures
How is FA Initially managed?
Refer for genetic counseling.Testing of siblings. Renal ultrasound, hearing test, eye exa,. Endocrine evaluation (check growth hormone levels, TSH). Bone marrow biopsy
What are FA treatment options?
Transfusions. Androgens (oral oxymethalone) can improve blood counts.Growth factors. BMT. FA cells are very sensitive to radiation and alkylating agents – can use greatly reduced doses
describe Embryonic stem cells
come from a five to six-day-old embryo. They have the ability to form virtually any type of cell found in the human body.
describe Embryonic germ cells
derived from the part of a human embryo or fetus that will ultimately produce eggs or sperm (gametes).
describe Adult stem cells
undifferentiated cells found among specialized or differentiated cells in a tissue or organ after birth. have a more restricted ability to produce different cell types and to self-renew.
What are potential graft sources?
Allogeneic: from another person. Syngeneic: from an identical twin. Autologous: from the patient. Umbilical cord blood
Describe Autologous Transplant use and its effects
No evidence of disease in the blood or bone marrow
Transplant related mortality (TRM) lowest with autos (<5%). Relapse rates are higher. Absence of graft versus host effects
Describe Allogeneic Transplant use and its effects
High TRM (30-50%). Lower relapse rates. Graft versus host effects
Describe the use of Umbilical Cord Blood and its effects
Cryopreserved, Small number of stem cells. Higher incidence of engraftment failure. Lower risk of GVHD. Degree of matching not as stringent. used for myeloablative or nonmyeloablative conditioning
What are Early complications of stem cell transplants?
Mucositis, Sinusoidal obstructive syndrome (VOD), Fluid retention, jaundice, hepatomegaly, Transplant related infections, Damage to mouth, gut and skin, Prolonged neutropenia
What are Delayed complications of stem cell transplants?
Chronic GVHD, Scleroderma or Sjogrens syndrome, Bronchiolitis, Keratoconjunctivitis, Malabsorption, Cholestasis, Esophageal stricture
What are Late complications of stem cell transplants?
Secondary Tumors (Acute leukemias, solid tumors, MDS). Late Infections-Need repeat vaccinations Pneumovax, Hep B, Hemophilus influenza b, poliovirus, diphtheria/tetanus, flu
