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An Indolent dz characterized by an overproduction of functionally incompetent mature B lymphocytes, which are smaller than normal and long lived.
Chronic lymphocytic leukemia CLL
What is the most commonly occurring adult leukemia?
What are the signs and symptoms of Leukostasis?
Respiratory distress, headaches, blurred vision, lethargy, priapism, slurred speech, confusion
Smudge cell is found in what?
Chronic lymphocytic leukemia, CLL
3 rationales for bone marrow transplantation
- 1. Elimination or eradication of diseased, or porely functioning bone marrow
- Corrolary is that the diseased marrow is replaced with healthy functioning marrow
- 2. Tumor Kill: Allows us to give very high doses of chemotherapy without worrying about marrow toxicity
- 3. Establish a clean slate for new marrow to take hold and fight remaining tumor
Diseases commonly treated by bone marrow transplant
Leukemia, lymphoma, multiple myeloma, severe aplastic anemia, fanconi anemia, dyskeratosis congenital, hemoglobinopathies (beta thalassemia, sickle cell), meylodysplastic and myeloproliferative disorders, germ cell tumors such as testicular cancer
True or False you can use nonmyeloablative therapy with an autologous transplant
False if you have an autologous transplant you have to kill with a full myeloablative therapy
High dose of chemotherapy and or radiation that is used for tumor kill, full marrow ablation.
This ablation technique absolutely requires! Stem cell rescue
What type of ablation is used for tumor kill
What type of ablation is used for a graft vs tumor effect?
Both myeloablative and nonmyeloablative
Another name for reduced intensity chemotherapy or mini allo
This technique uses lower doses of chemotherapy to create a platform for stem cell transplant
Type of bone marrow transplant using an Identical twin�s bone marrow or peripheral stem cells
Synergetic Allogenic stem cells
Type of bone marrow transplant using a donor with a HLA identical sibling/relative
Matched related allogenic
Related donor is not HLA identical to the recipient
Mismatched related allogenic
What are the three types of Related allogenic transplants?
- Matched related
- Mismatched related
What are the 3 types of unrelated allogenic transplants?
- Mismatched unrelated
- Matched unrelated (MUD)
- Cord blood
Donor is HLA identical to an unrelated person
Donor is not HLA identical to the recipient and is unrelated
Donor stem cells are take from an umbilical cord immediately after delivery of an infant
HLA (MHC) is found on chromosome __
Group of genes that relate to the immune system
4 factors considered when deciding what type of transplant and conditioning to give the recipient
- patient diagnosis and disease stage
- patients age
- degree of donor-patient match called HLA typing
What are the 2 types of Lymphoma
Hodgkins and non-Hodgkins
What are B symptoms?
Fever, night sweats (and day sweats), weight loss
Are B symptoms more common in NHL or Hodgkins disease?
What types of NHL are treated with BMT? Not specific disease types of NHL
- Chemo-sensitive disease in the first relapse
- Low grade disease transformed into high grade
- Standard treatment for relapsed disease
- Aggressive lymphoma
BMT may be used upfront for patients with high risk disease, which has one or more of what 4 qualities?
- Age > 60
- High LDH
- Stage 3 or 4
- More than one extranodal site
Chronic inflammatory diseases such as Sjogren�s syndrome, celiac disease and RA are associated with what types of cancer?
B cell and T cell neoplasms
What are the two indications for BMT in patients with Hodgkin�s disease?
- Patients who do not achieve a remission with first line therapy
- First or subsequent relapse of disease
Hodgkin�s disease is CD ___ and CD ___ positive
AML is a disease of (young or old) people?
What is standard induction therapy for AML?
- 3+7 � daunorubicin for three days and cytarabine for 7 days
- or 5 and 2 for older patients
What are some prognostic that determine factors for overall survial in AML?
Age, WBC >100K, preexisting disorder (MDS,chemo,radiation), and Cytogenetics (favorable, t8,21, inv6 poor, +8,-7,-5 or multiple aberrations)
Plasma cell cancer
What is the standard treatment for Multiple myeloma?
Bone marrow transplant once disease burden has been reduced
When would you use BMT to treat a Germ cell tumor (testicular cancer) ?
If they only have partial response to therapy or refractory disease
Testicular cancer typically affects men of what age?
What types of diseases are treated with Autologous stem cell transplant?
Lymphoma (both NHL and HD), Multiple myeloma, Acute myeloid leukemia, Germ cell tumors
Stem cell collecting process from peripheral blood is called what?
Dilantin, sulfonamides, penicillin and hydralazine can all cause this symptom that mimics Lymphoma
Rapid progressive lymphadenopathy is a sign of _____ while waxing and waning lymphadenopathy is a sign of ____
Aggressive lymphoma, indolent lymphoma
3 Characteristics of Lymphadenopathy suggestive of lymphoma
- significant size (greater than 1.5 by 1.5 cm)
- persistence for more than 4 wks
- progressive increase in size
What type of biopsy is best for suspicious lymph nodes?
On pathology report of a lymph node you note the pathogist decribes a very large cell with abundant pale cytoplasm and two or more oval lobulated nuclei containing large nucleoli and it appears red on H+E stain. You know instantly it is what type of cancer?
It�s a Reed Sternberg cell indicative of Hodgkin�s disease
What types of disease are treated with Allogenic stem cell transplant?
Leukemia (AML, ALL, CML), myelodisplastic syndrome, myeloproliferative disease, marrow failure states, myeloma, lymphoma
What are some Indications for Adult allogenic Transplant?
AML, ALL, Myelodysplatic syndromes (pre-leukemia), CML, Lymphoma, Multiple Myeloma, Bone marrow failure states
What are some indications for pediatric allogenic transplant?
Hematologic Malignancies, hematologic disease or disorder, Immunodeficiencies, Genetic or metabolic disorder
What are 4 reasons for increased treatment related mortality in older patients undergoing myeloablative transplantation?
- Comorbid medical conditions- renal, cardiac, pulmonary
- Complications of treatment prior to transplant
- Increased incidence of graft vs. host disease after transplantation
- Advanced age- treatment mortality and relapse rate are higher in older people
What are 4 advantages of non-myeloablative treatment as opposed to myeloablative transplantation?
- Less treatment related toxicity
- Co-morbid disease is less of a barrier to transplantation
- Less graft vs host disease
- Less cost and improved quality of life
What is the main drawback of non-myeloablative therapy?
Increased incidence of relapse
What are the 3 parts (1/3rds) of the Transplantation process?
- Conditioning (chemotherapy)
- Stem cell infusion and supportive care (neutropenia, complications and sx management)
- Recovery and engraftment
What are some common side effects of Conditioning therapy?
Drug reactions, mucositis, nausea, vomiting, fatigue, alopecia, rash, organ damage (pulmonary toxicity, venooclusive disease, renal failure)
SEs of cyclophosphamide
Cardiovascular, genitourinary, endocrine
SEs of Busulfan
Neurotoxicity, delayed pulmonary toxicity
Name some medications that are used to prevent graft vs host disease?
Tacrolimus, sirolimus, rapamycin, methotrexate
Donor cells attack the healthy cells of the recipient patient
Graft vs host disease
Sx of GvHD
Maculopapular rash, nausea, vomiting, anorexia, profuse diarrhea, ileus or cholestatic hepatitis and occurs within 100 days of transplantation or DLI
T or F patients who develop acute GvHD will not develpe chronic GvHD later
F. they are actually at higher risk of cGvHD