Bone Marrow Disorders Flashcards
What is Pure Red cell Aplasia (PRCA)?
-aka
- erythroblastopenia
- type of anemia affecting the precursors to red blood cells .
- severe reduction in the reticulocytes in peripheral blood
- virtual absence of erythroid precursors in bone marrow
- bone marrow often ceases to produce RBC
Causes of Pure Red Cell Aplasia?
- autoimmune disease
- Thymoma
- Viral infections such as HIV, herpes, parvovirus B19
- Large granular lymphocyte leukemia
- Idiopathic
- Congenital: “hereditary pure red cell aplasia” has been used to refer to Diamond Blackfan anemia
Pure Red Cell Aplasia Dx is suspected in a pt with:?
It is confirmed with?
- Dx is suspected in a pt with:
- anemia and very low reticulocyte count
-confirmed with: normocytic normochromic anemia -normal WBC and platelets -normocellular bone marrow -reticulocyte count
Pure red cell aplasia Tx
- red cell transfusion for symptomatic anemia
- cessation of possible drugs causing this disease
- immediate hematologic consultation
What is the prognosis of Pure Red Cell Aplasia?
- remitting and relapsing course requiring long-term support of their anemia with red cell transfusions
- long term tx with immunosuppressive or cytotoxic agents
Myelophthisic Anemia, what is this?
- severe kind of anemia found in ppl w/ diseases that affect the bone marrow.
- the displacement of hemopoietic bone-marrow tissue into the peripheral blood either by fibrosis, tumors, or granulomas.
What causes Myelophthisic Anemia?
- chronic myeloproliferative diseases (myelofibrosis)
- leukemia
- lymphoma
- Metastatic carcinoma or myeloma (breast, lung, or prostate cancer that has metastasized to bone marrow)
How do you diagnose Myelophthisic Anemia?
- otherwise unexplained quantitative changes in one or more of the blood and bone marrow elements.
- Blood smear; erythrocytes that contain nuclei or dacryocytes (tear drop cell)
- leukoerythroblastosis; the displaced hematopoietic cells begin to undergo extramedullary hematopoiesis
- blasts account for less than 20% of total cells of bone marrow aspirate and peripheral blood, cases with higher blasts are considered to have acute myeloid leukemia
*** DIagnosis confirmed when bone marrow biospy demonstrates significant replacement of bone marrow by fibrosis, malignancy, or other infiltrative process.
***MDS is a diagnosis of exclusions
Myelophthisic Anemia Tx?
-treat the underlying cause/cancer
- consider bone marrow transplant in young
- supportive (RBC transfusions, treat infections)
- Growth Factors (EPO)
Myelodysplastic Syndrome (MDS) what is this?
-variable reduction in the production of normal red blood cells, platelets, and mature ganulocytes(neutro, eosino, baso)
Myelodysplastic Syndrome leads to what type of systemic consequences?
- anemia
- bleeding
- increased risk of infection
What causes Myelodysplastic Syndrome?
- most cases are idiopathic
- environmental factors (chemicals, radiation, tobacco, chemo drugs)
- Gene abnormalities (trisomy 21, blooms syndrome, Fanconis Anemia)
- other hematologic diseases (paroxysmal nocturnal hemoglobinuria, congenital neutropenia)
40% of the time what does Myelodysplastic syndrome result in?
-acute leukemia
Clinical and laboratory manifestations of Myelodysplastic Syndrome?
-Anemia: macro, normocytic, oval macrocytes on peripheral smear
- neutropenia
- thrombocytopenia
- purpura: bruising w/ thin skin in old age in sun exposed areas.
What is the only potentially curative therapy for MDS patients?
bone marrow therapy
What are some indicators of a good prognosis of MDS? Indicators of poor prognosis?
- younger age
- normal or moderately reduced neutrophil or platelet count
- low blast counts in bone marrow
- no auer rods (red staining needle like bodies seen in cytoplasm of myeloblasts)
- advanced age
- severe neutropenia, thrombocytopenia
- high blast count
- auer rods
Stem Cell definition
- a cell that has the ability to continuously divide and differentiate into various other kind(s) of cells/tissues
Kinds of Stem cells
- embryonic stem cells: come from 5-6 day old embryo. can form virtually any type of cell in the body.
- embryonic germ cells: derived from part of a human embryo or fetus that will ultimately produce eggs or sperm
- 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
Why are Hematopoietic Stem cell transplants performed?
Where are hematopoietic stem cells retrieved from?
- to re-establish hematopoietic function in patients w/ damaged/defective bone marrow or immune systems.
- potentially curative for a wide variety of disorders.
- collected from the bone marrow, peripheral blood, or umbilical cord blood.
What are the types of grafts?
- allogenic: from another person
- syngeneic: from identical twin
- autologous: from the patient
- umbilical cord blood
Consequences of Autologous Transplant?
-whats one requirement you must meet to have autologous transplant?
- transplant related mortality is lowest
- relapse rates are higher depending on disease
- absence of graft versus host effects
Requirement:
-no evidence of disease in the blood or bone marrow
Consequences of Allogenic transplant?
- High transplant mortality
- lower relapse rate due to graft vs tumor effects
- graft vs host effects (transplant attacks host cells)
What is the chance (%) of having a sibling match for a graft?
25%
Consequences of Matched unrelated donors in transplant?
- severe graft vs host disease
- Higher transplant related mortality
Who are considered haploidentical donors?
-from parent, child, or sibling
Umbilical cord blood:
how is this stored? How is cell dose given?
- obtained from umbilical vein and frozen with an anticoagulant and nutrient media.
- per recipient weight
How are stem cells collected?
- Bone marrow harvest: general anesthesia
- 2 deaths in 8000 collections?
- stem cells circulate in the blood, identified by CD34+ by flow cytometry, colleced through apheresis catheter.
- higher GVHD than bone marrow harvest
- more rapid marrow recovery
How long can we wait before infusing stem cells?
may be used fresh within a few hours of collection or frozen using DMSO
Early Complications of Stem Cell/graft
- mucositis (painful inflamm and ulceration of mucous membranes lining the digestive tract)
- sinusoidal obstructive syndrome (VOD; veno-occlusive disease) fluid retention, jaundice, hepatomegaly
- transplant related infections– damage to mouth, gut, skin, prolonged neutropenia, pnacytopenia (deficiency of RBC, WBC, and platelets)
- Graft vs host Disease, graft rejection, interstitial pneumonitis
Delayed complications of stem cell/graft ?
- Chronic GVHD
- secondary tumors (Acute leukemias)
- late infections: bacterial, viral, fungal
