Hem Malignancies

Question 1

Overview of Hematological Malingancy Categorization and Nomenclature

Answer 1

Leukemia: white blood: too many cells in blood
Lymphomas: masses in lymph nodes or soft tissues
Diff is: Leukemias are liquid, lymphomas are masses
Acute Leukemia: lethal in weeks wo tx. More sensitive to chemo.
Chronic: may survivie many years without tx.

Question 2

Differentiate between Lymphoid and myeloid malignancies

Answer 2

Myeloid: give rise to granulocytes, RBCs, platelets, etc.
Lymphoid: give rise to B cells, T cells, NK cells, etc.

Question 3

Describe the nomenclatures of acute leukemias

Answer 3

2 types:
Acute Myeloblastic L: from granuloctyes, RBC, platelet
- Most common in adults,
- FAB catergorization: M0-M7 based on morphological apprearance.
- WHO classification: to describe the type

Acute Lymphoblastic L: From B, T, NK cells

• Common in children
• Two types B-ALL and T-ALL
• more aggressive.

TUmor cells develop in teh marrow and suppress other cells in marrow.

Question 4

Define myeloproliferative diseases and identify clinical and genetic differences amongst these diseases

all these are from myeloid stem cells

Answer 4

Chronic Myeloproliferative: dz where marrow makes too many RBCs, platelets, or WBCs.

Polycythemia Vera:

• JAK2 mut
• HIgh levels of RBCs
• Survival less than 1 yr,
• Tx: Periodic phlebotomy/chemo

Essential Thrombocythemia

• JAK2 or MPL mut
• Too many Megakaryocytes
• Asymptomatic until late life

Primary myelofibrosis

• JAK2 ro MPL mut
• Fibrosis and atypical Megakaryocytes
• 1-8 yrs survival

Chronic Myelogenous Leukemia

• BCR ABL gene mut
• Too many granulocytes and megakaryocytes
• untreated progress to Acute leukemia in 5 yrs
• Tx: Imatinib or stem cells

Question 5

Describe the major divisions in classification of lymphomas

all these are from Lymphoid stem cells

Answer 5

Hodgkins Lymphoma:
+ Reed Sternberg Cells (huge cells with multinucleus)
- usually in one lymph node region and spread predictably
- Tx: radiation/chemo w/ good cure rate

Non Hodgkins Lymphoma:

• Reed sternberg Cells
• Follicular lymphoma - Germinal center b cell, BCL-2 mut, indolent
• Extranodal marginal zone lymphoma, post germinal center b cell, NFkB mut
• Diffuse large B cell lymphoma - Germinal center, BCL-6, BCL-2, aggressive
• Burkitt Lymphoma - Germinal center b cell, c-MYC, very aggressive, EBV

Chronic Lymphocytic Leukemia

• Not assoc with single gene mutation - mulit factorial
• older people
• smudge cells - fragile
• pnts asysmptomatic at dx

Question 6

Identify key clinical features and laboratory values that allow for differentiation of PLASMA CELL DISORDERS

Answer 6

Multiple Myeloma

• comp of Mature B cells (plasma cells)
• Rouleaux formation - stacked RBCs
• M prot in urine/serum
• Lytic bone lesions

Solitary Plamacytoma

• Big mass of clonal plasma cells
• Low levels of Monoclonal antibodies (M prot) in serum
• Risk of progression to Multiple Myeloma

Primary Amyloidosis

• tons of plasma cells that produce monoclonal LIGHT CHAINS, which are misfolded and form deposits
• cause organ prob

Monoclonal Gammopathy of Uncertain Significance (MGUS)

• see monoclonal immunoglobin prot in serum/urine without evidence for malignancy of plasma cells or b lymphocytes.
• can progress to multiple myeloma

POEMS syndrome - Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, Skin changes

Question 7

Overview of common diagnostic techniques used for hematological malignancies

Answer 7

Leukemias: Peripheral blood smear, bone marrow biopsy
Lymphoma: lymph node biopsy
Plasma Cell neoplasm: Bone marrow biopsy

Morphology: sending sample for staining

• AML: auer rod seen
• Mult My - homogenious cells in marrow

Immunophenotyping via flow cytometry.

HIstochemistry: biopsy - to see if ab markers present.

Cytogenetics: karyotype, FISH

Serum protein elecrophoresis

Question 8

What are key chemotherapeutic terms associated with treatment of hematologic malignancies?

Answer 8

Induction therapy: Initial tx given for cancer. goal is to induce remission of dz by reducing number of cells.

Consolidation therapy: Tx given following induction therapy, goal is to eliminate any undetectable cancer cells

Maintenance therapy: Therapy given after pnt has acheived remission. goal is to maintain remission and reduce risk of relapse.

Adjuvant therapy: given with prmary therapy.

Palliative therapy: lessens the symptoms adn improve QOL

Salvage therapy: Tx given after others have failed.

Question 9

Describe Myelodysplastic syndrome

Answer 9

Not making enough blood cells. can progress to acute myeloid leukemia

Question 10

Remember to look at the charts shown in lectures to help organize

Answer 10

Yeah!!