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Flashcards in Heme/onc part 2 Deck (44):

What are more than 90% of the cases of CML due to?

Philadelphia chromosome
Translocation of the long arm of chromosome 22 and 9
Results in the shortening of chromosome 22


Presentation of CML

Usually discovered incidentally with leukocytosis on CBC
Many times pts will have splenomegaly on PE
CML can transform into acute leukemia, will see an increase in blasts and new chromosomal abnormalities


S/sx of CML

Low-grade fever
Decreased appetite
Chronic fatigue
Wt loss
Excessive sweating


CBC findings of CML

Elevated WBC (20,000-60,000)
Lymphocyte count will be nl
Will usually see an increase in other granulocytic cells (basophils, eosinophils, etc)


Other lab findings of CML

Could have low or absent ALP
Leukocyte alkaline phosphatase (LAP) stains very low to absent in most cells, resulting in a low score
BMBx: hypercellular (many myeloid cells seen, such as basophils, neutrophils, eosinophils)


Stage of CML

Dz is biphasic, sometimes triphasic
Chronic phase
Accelerated phase
Acute phase (blast phase)


Tx of CML

Gleevec is the 1st line chemotherapy. It targets the BCR/ABL gene by inducing apoptosis in cells pos for BCR/ABL
Usual dosage is 400 mg PO daily
Bone marrow transplant is currently the only known cure for the dz


When does ALL most commonly occur?

In childhood


S/sx of ALL

Joint pain
Wt loss
Persistent and frequent infections
Gingival bleeding
LAD and hepatosplenomegaly is common in ALL, as the lymphocytes are the involved cell lineage



Gingival edema
Chloroma (mass of leukemic cells found outside of the bone marrow)
Testicular edema


Lab evaluation of ALL

CBC will reveal elevated WBC with lymphocytosis
Bone marrow bx will reveal leukemic blasts
CXR may revieal a mediastinal mass
Cytogenetic studies
-Translocation 12:22; most common and favorable prognosis
-Philadelphia chromosome is unfavorable prognosis in ALL


Tx of ALL

Induction and consolidation chemotherapy
>50% of children will be cured with chemo
Prognosis depends upon WBC and age at the time of dx


Chronic lymphocytic leukemia

Most common adulthood leukemia
Affects the B-cell lineage


Characteristics of CLL

Most pts are asymptomatic when discovered
CBC reveals leukocytosis, with predominant lymphocytes
-Must persist for >3 mos for dx


Further evaluation of CLL

Peripheral blood flow cytometry: Usually express CD19
FISH for CLL and cytogenetic studies: evaluates chromosomes 6, 11, 12, 13, 14, 17, 18, or 19
-Used as a prognostic indicator
If significant LAD present, needle bx should be obtained


Peripheral blood smear of CLL

Smudge cells present


S/Sx of CLL

Enlarged lymph nodes, liver, or spleen
Recurring infections
Loss of appetite or early satiety
Abnl bruising (late-stage sx)
Night sweats


Tx of CLL

Early stage CLL is monitored closely. Routine CBCs performed q3-6 mos. Flow cytometry is performed about q6 mos
-Absolute monoclonal B-lymphocyte count <5000
-Lymph nodes <1.5 cm
-No anemia or thrombocytopenia
As dz progresses, chemo is necessary
-FCR is generally 1st line tx in pts <70 yo
--Fludarabine, cyclophosphamide, Rituxan


How are NHL tumors characterized?

By the level of differentiation, the size of the cell of origin, the origin cell's rate of proliferation, and the histologic pattern of growth


S/sx of NHL

B sx
-Night sweats
-Wt loss


Low-grade NHL

Generally are felt to be slow-growing indolent diseases
Treatable with chemo but not curable
Pathologically described as grade 1 follicular or small lymphocytic lymphomas
Typically older pts- usually feel well, generally have no sx, and often have their dz found incidentally in the workup of another medical problem
Pts can often live up to 10+ years without requiring tx with chemo


Intermediate grade NHL

More aggressive
Can be found in both young and older pts
They are usually grade 2 or 3 and consist of the more common subtypes
-Diffuse large B cell, mantle cell, and follicular lymphomas
Pts usually are experiencing some or all of the B sx
This is very curable, even in pts with stage 3 or 4 dz
Tx should be started ASAP


High grade NHL

The most aggressive
Generally present in the younger, adolescent pt population
Pathologically, they include types such as Burkitt's lymphoma, T-cell lymphomas, and anaplastic lymphomas
CNS involvement can commonly occur
Pts are often symptomatic at the time of presentation, and again, tx should start ASAP


Tx of low-grade NHL

Chemo initiated with progressive dz and bulky LAD
Usually tx with CHOP: Cyclophosphamide, hydroxydaurnorubicin, Oncovin, prednisone
-Chemo treatments do not have to be as aggressive in this situation


Adjuvant tx to chemo in NHL

Rituxan, which is targeted therapy to inactivate CD20+ lymphoma cells
Given with the second cycle
Often used once chemo is complete as maintenance therapy is given every 3-6 mos


Tx of intermediate/high grade NHL

If early stage I or II dz, can sometimes treat with radiation only, depending on type of lymphomas and site of involvement
Otherwise, will need to treat with chemo +/- Rituxan


Characteristics of Hodgkin's lymphoma

Characterized by Reed-Sternberg cells
EBV is related in 40-50% of cases
Usually arises in one lymph node group and spreads to other nodes


Subtypes of Hodgkin's lymphoma

Nodular sclerosis
Mixed cellularity
Lymphocyte depleted
Lymphocyte rich


Incidence of Hodgkin's lymphoma

Bimodal incidance of Hodgkin's with the first peak in pts in the twenties and the second peak after the age of 50
Most children with the disease are males


Clinical features of Hodgkin's lymphoma

`Painless cervical, supraclavicular, and mediastinal LAD
B sx
Nodular sclerosis subtype is usually seen in young women


B sx

Night sweats
Intense pruritis
Unexplained fever
Unintentional wt loss
Fatigue and generalized weakness


Tx of Hodgkin's lymphoma

Early stage disease I or II can often be treated with radiation alone or a combination of short course chemo and involved field radiation therapy
Stage III or IV dz is treated with chemo
If there is bulky mediastinal involvement, radiation to the chest may also be done


What should be done for pts that relapse after first line Hodgkin's lymphoma tx?

Additional chemo is recommended along with auto stem cell transplant which can also afford long term survival and possible cure


What is the most common hematologic malignancy in the African American population?

Multiple myeloma


What is oftentimes the first presentation of multiple myeloma?



Lab work for multiple myeloma

Chemistries- including total protein, albumin, and calcium levels
Immunofixation studies from serum and urine
Immunoglobulin levels- IgG
Skeletal survey
Bone marrow bx
Radiographic studies to evaluate specific areas of pain


Lab findings of multiple myeloma

Routine CBC may often shown anemia and/or thrombocytopenia
High total protein level, high calcium and an elevated creatinine
Skeletal survey will show classic signs of lytic lesions in the bone
A bone marrow bx that shows more than 20% plasma cells is usually diagnostic for multiple myeloma


Stage I multiple myeloma

Pts must have all of the following along with a bone marrow bx showing <20% plasma cells:
-Hgb >10
-Serum calcium nl or <12
-Relatively small amount of monoclonal immunoglobulin in the serum or urine
-Skeletal survey nl or with solitary plasmacytoma


Stage II

Pts who qualify for neither stage I nor III
-A moderate number or myeloma cells are present


Stage III multiple myeloma

Pts that have any one of the following along with bone marrow bx showing >20% plasma cells
-Hgb <8.5
-Serum calcium >12
-High M component in serum
-UPEP M component >12 gm/24 hr
-Extensive lytic lesions


Tx of multiple myeloma

High doses of decadron is still a mainstay of tx
-Combined either with oral biologic agents such as Thalidomide or Revlamid or intravenous chemo agents
Some pts may qualify for high dose chemo and autologous stem cell transplant, depending on their age and extent of dz


Tx of hypercalcemia and lytic bone lesions in multiple myeloma

IV chemo agents and bisphosphonates
Usually Zometa 4 mg IV q4 wks


Kyphoplasty in multiple myeloma

A procedure done by neurosurgeons or orthopedic surgeons to stabilize vertebral bodies that have been compromised


F/u of multiple myeloma

Pts should be monitored every 2-3 mos with repeat lab work to determine response to dz
-A repeat bone marrow bx would be indicated if new lab abnormalities were to develop