Leukemia, Lymphoma, and Multiple Myeloma Lecture Powerpoint Flashcards
Induction chemo vs consolidation chemo
Chemo therapy used to reduce tumor load without intention of cure vs used to induce a remission
Remission
Disease free but not necessarily a cure
Intensification
Chemotherapy after complete remission with higher doses of chemo with intent of increasing cure rate or prolonging remission duration
Zubrod scale
A scale from 0 (normal), 1 (symptomatic and ambulatory), 2 (ambulatory 50% of time and need occasional assistance), 3 (ambulatory less than 50% of time nursing care needed), or 4 (bedridden) to analyze performance status of cancer patient
Flow cytometry
Lab test of peripheral blood or bone marrow aspirate that can identify circulating myeloblasts in the majority of patients by characteristic patterns of surface antigen expression, used in most hematological malignancies to make diagnosis
Gold standard for diagnosis of leukemias
Bone marrow biopsy and aspiration of marrow
Is thrombocytopenia a contraindication to bone marrow biopsy and aspiration of marrow?
No
Prechemo evaluation protocol (6)
- Check for history of CHF (contraindication)
- HSV, CMV, HBV, HCV, HIV status
- HLA typing
- 2D echocardiogram
- Dental eval
- Central line placement
Drug choices to alleviate nausea and vomiting as a side effect of chemo (3)
- Dexamethasone
- lorazapam/ativan
- haldol
Drug choices to alleviate pancytopenia as a side effect of chemo (2)
- Neupogen (filgastrim)
- epogen (procrit)
Drug choices to alleviate diarrhea as a side effect of chemo (4)
- Imodium
- Octreotide
- Questran
- lomotil
The absolute neutrophil count calculation
Total WBC x (% of segs + % of bands)
Febrile neutropenia
A patient that has completed chemo or is still undergoing chemo that develops a fever (has ANC <1500) and are thus very susceptible to infections from various sources
Evaluation and treatment of febrile neutropenia
- Good physical exam (look at skin sites and catheters)
- culture
- CXR
- Cefepime (tx pseudomonas), antifungal (diflucan), antiviral (acyclovir)
Tumor lysis
A treatment complicationn of cancer where tumor cells being lysed by chemotherapeutic agents results in large release of K+, pohsphate, and nucleic acids into systemic circulation, occurs in rapidly dividing cancers and patients with large tumor load and can have severe effects such as NVD, lethargy, heart failure, renal failure, DIC, and sudden death
Prevention of tumor lysis (3)
- pre chemo hydration
- allopurinol/zyloprim
- careful monitoring
Autologous vs autogenic hematopoietic cell transplant
-Either an autotransplant (requires patient not to be bed ridden and have good function) or donor based (harder to match up)
Fertility preservation and hematologic cancer patients
Goal to discuss fertility issues prior to starting treatment for cancer, sometimes treatment requirements do not allow time to collect ovum or sperm
WHO classification of the hematopoietic neoplasms
-Based on combo of morphology, immunophenotype, genetics, and clinical features
Acute vs chronic leukemia
- Acute usually means hospitalization and immediate treatment (IV chemo and transfusions), fast growing, post chemo complications more frequent and severe in acute dz
- Chronic usually means outpatient treatments and many treatment options use pills opposed to IV, slow growing,
Acute myelogenic leukemia (AML)
-80% of all acute leukemias, most frequent in neonates, result of proliferation of leukemic cells in myeloid line interfering with production of normal blood cells causing weakness, infecttion, and bleeding. Generally rapidly lethal unless treatment with intensive chemo or other targeted therapy
Environmental factors of acute myelogenic leukemia (AML) (3)
- exposure to high dose radiation
- retroviruses
- Tobacco use
Familial disorders as cause of AML (rare) (5)
- down syndrome
- bloom’s syndrome
- fanconi’s anemia
- aplastic anemia
- myeloproliferative disorder
Presenting symptoms of AML (4)
- fatigue (most common)
- fever (from infection)
- extramedullary disease
- leukemia cutis on skin
Auer rods
Lab abnormalities of AML present in 50% of cases, can also occur in myelodysplastic disease
AML treatment standard
7+ 3 regimen, (7 days of cytabine and 3 days of daunorubicin)
Pregnant patient with AL
If found during 1st trimester must immediately terminate pregnancy and start treatment, delay treatment in 2nd and 3rd trimester till post partum if possible using transfusions with induction of labor and delivery of viable fetus as soon as possible
Chemo is not used in patients over age of __ regardless of health status as it will worsen patient outcome
..80
Leukopheresis
Removal of excessive WBC from peripheral blood in AML treatment
CNS leukemia
Occurs rarely as side effect of AML, diagnosed via LP, cranial nerves III and VI affected most commonly
Acute promyelocytic leukemia (APL)
A form of AML that is an emergency, occurs with presence of atypical promyelocytes in bone marrow and peripheral blood (have creased fold or dumbell nuclei), worrisome to avoid hemorrhage from characteristic coagulopathy (pulmonary or cerebrovascular hemorrhage) (mean survival <1 month if left untreated)
APL treatment (2)
- All trans retinoic acid (ATRA)
- 7+3 regimen (as a baseline in suspected APL)
Differentiation syndrome (cytokine storm)
Occurs in 25% of APL patients within days of starting ATRA + chemo regimen characterized by fever, peripheral edema, pulmonary infiltrates, hypoxemia, respiratory distress, hypotension, etc.
Differentiation syndrome (cytokine storm) treatment (1)
-Dexamethasone for 3 or more days
ATRA Treatment complications (2)
- hyperleukocytosis (leukophoresis can worsen APL)
- High intracranial pressure
Chronic myelgenous leukemia (CML
Dysregulated production and uncontrolled proliferation of mature and maturing myeloid cells with fairly normal differentiation, presents with fatigue, malaise, hallmark is absolute basophilia (basophils >20% on diff), has a triphasic course
Leukemoid reaction
High WBC with neutrophilia and prominant left shift usually seen with infection and might be initially misinterpreted as leukemia
Triphasic course of untreated CML
1) Chronic phase - usually asymptomatic, 3.5-5 years before transforming into aggressive phase
2) accelerated phase - B symptoms appear (fever, sweats, weight loss 10% in 6 months), sometimes skipped
3) Blastic phase - morphologically resembles acute leukemia, usually die within 6 months without treatment
Philadelphia chromosome
BCR-ABL fusion gene, occurs in 95% of CML diagnoses and diagnostic for CML
CML treatment (2)
- Allogenic hematopoietic cell transplant (curative, used if genetics unfavorable for TKI treatment)
- tyrosine kinase inhibitors (TKI) (control)
Imatinib/gleevec
A tyrosine kinase inhibitor that is NOT curative but helps push remission that targets specific abnormal tyroosine kinase made from cancer causing gene used to treat CML, does not attack normal cells and can be used orally with minimal side effects such as fluid retention
Acute lymphoblastic leukemia/lymphoblastic lymphoma (ALL/LBL)
A group of hematopoietic neoplasms most common childhood malignancy (25% of all child cancers) involving the cells that form the lymphoid lineage, can occur with or without the philadelphia chromosome, divided into T cell and B cell versions, called leukemia if >25% bone marrow blasts, called lymphoma if <25% bone marrow blasts, is frequently missed due to non-distinct symptoms
Risk factors for ALL/LBL (6)
- advanced paternal age
- maternal fetal loss
- down syndrome
- neurofibromatosis
- bloom syndrome
- ataxia telangiectasia
Bloom syndrome
Rare recessive genetic disorder characterized by short stature, predisposition to cancer development, and general genomic instability
ALL/LBL presentaiton in children vs adults
- Children see fever, hepatomegaly, and general malaise
- Adults see pain, mediastinal mass
Hyper CVAD
A chemotherapeutic regimen of cytoxan, vincristine, daunorubicin, and dexamethasone used for ALL/LBL without philadelphia chromosome
ALL/LBL treatment options (3)
- Hyper CVAD
- TKI if philadelphia chromosome pos
- prophylactic CNS treatment (Neupogen granulocyte colony stimulating factor)
Diagnosis of CNS ALL/LBL
-LP and obtain CSF
Ommaya reservoir
A surgically placed tube going from the scalp deep into the ventricles for administration of drugs or alternative to LP to withdraw CSF
New treatments for relapsed or refractory B cell ALL
Chimeric antigen recetpor T cells (CAR-T)
Chronic lymphoblastic leukemia/small lymphocytic lymphoma (CCL/SLL)
Progressive accumulation of funcitonally incompetent lymphocytes which are usually monoclonal in origin, CLL is used when primarly manifests in blood, SLL when primarily nodal
Most common leukemia in western world and what is unique to it?
Chronic lymphocytic leukemia (CLL), it is the only leukemia NOT associated with exposure to radiation
CLL/SLL presentation (3)
- Waxing and waning painless lmphadenopathy often in cervical area walders’s ring
- acquired immunodeficiency disorder
- B symptoms
Diagnosis of CLL
- Absolute lymphocyte count in peripheral blood >5000
- demonstration of clonality of the circulating B lymphocytes by flow cytometry
Richter’s transformation
A rare occurrance in CLL patients with a transformation to highly aggressive non hodgkin lymphoma with poor prognosis
CLL Treatment (3)
- NOT curable
- Localized radiation therapy to lesions
- advanced undergo chemo, no single agreed upon regimen
Lymphoma
Group of cancers in lymphoid tissues derived from B or T cell progenitors or mature that undergo malignant change and begins to multiply crowding out healthy cells creating tumors which enlarge nodes, either non hodgkin (more common) or hodgkin
Presentation of nonhodgkin lymphoma (NHL)
-Aggressive lymphomas that present acutely or subacutely with B symptoms, often insidious
NHL complications (4)
- epidural spinal cord compression
- pericardial tamponade
- vena cava obstruction
- intestinal obstruction
Lugano classification
Staging of lymphoma based on involvement of one lymph node (I) group, stage II involvement of 2 or more lymph node groups on ssame side of diaphgragm alone, stage III involvement of lymph node systems on both sides of diaphragm, stage IV extensive extranodal disease
NHL diagnostic studies (3)
- lymph node biopsy
- CT
- PET scan
Hairy cell leukemia (HCL)
A B cell lymphoproliferative disorder, accumulation of small mature B cells with abundant cytoplasm hairy projections in peripheral blood, bone marrow, and splenic red pulp, unknown pathogenesis but can be familial,very insidious
Hairy cell leukemia symptoms (HCL) (3)
- fatigue, weakness (NO FEVER or NIGHT SWEATS)
- bruising or bleeding
- asymptomatic
Treatment of HCL (2) and prognosis
- Usually observation
- no curative treatment
- many undergo remission without issue
Diffuse large B cell (DLBCL)
An AIDS defining malignancy of nonhodgkin lymphoma
Burkitt lymphoma
Highly aggressive B cell tumor with 3 forms (endemic africa big mass on jaw bone, non endemic in US with abdominal presentation, AIDS), rapidly growing tumor with evidence of tumor lysis (doubling time 24 hrs)
R-CHOP regimen
Chemotherapy group of agents used in treatment of NHL
Rituximab (rituxan) use raises concern for reactivation of what pathogen if previously infected?
Hep B/C
Hodgkin lymphoma risk factors (4)
- standard of living
- infectious mononucleosis
- HIV
- lack of breastfeeding
Younger siblings of hodgkin lymphoma patients have __x the expected rate of risk, but not older siblings
7
Hodgkin lymphoma peak aged
-approx 20’s-30’s and 50’s
Reed sternberg cells
Indicative of some types of hodgkin lymphoma and have an owl appearance
Hodgkin lymphoma presentation (3)
- asymptomatic lymphadenopathy
- mediastinal mass on x ray
- generalized pruritis
- rubbery lymph nodes***
Hodgkin subtypes WHO classification system (2)
Classical: nodal sclerosis predominantly
Nodular lymphocyte predominant HL or folliclular HL
Hodgkin lymphoma treatment regimen for stage IIIB and IV
ABVD regimen + radiation
Hodgkin lymphoma long term treatment toxicities (3)
- Myelodysplastic syndrome
- cardiomyopathy
- infertility
Largest non cancerous cause of death in hodgkin survivors
Cardiac deaths
Test for multiple myeloma (3)
- S-PEP (serum protein electrophoresis)
- CT cross sectional imaging
- bone marrow biopsy
Myeloma
Malignancy of plasma*** cell which produce immunoglobins, when too much proliferation in the bone marrow occurs it can cause skeletal destruction and other pathological fractures
MM treatment (3)
- Initial treatment is needed immediately, patients will die within 6 months
- bone marrow autologous transplant
- chemo 3 drug regimen (bortezomib/velcade + lenalidomide/revlimid + dexamethasone/decadron)
Signs of multiple myeloma (4)
- Pallor
- anemia
- bone pain
- hypercalcemia
S-PEP and M spike
Serum protein electrophoresis - a technique that measures specific proteins in the blood, results see a “church steeple” of a significant proteins levels indicative of multiple myeloma (M spike)
Multiple myeloma CRAB criteria
One of the following that must accompany clonal bone marrow plasma cells >10% hyperCalcemia Renal insufficiency Anemia Bone lesions
“punched out lesions” on radiograph are indicative of…
….multiple myeloma
Monoclonal gammopathy of undetermined significance (MGUS)
Noncancerous condition with abnormal M protein that occurs with 1% progression to multiple myeloma, requires waitful watching but does not cause symptoms