11 Malignant disorders of WBC Flashcards
malignant disorders of WBC’s
1 leukemia
2 lymphoma
3 plasma cell myeloma
-common neoplastic disorders of bone marrow + lymphoid tissues
leukemia
- circulating tumors
- primarily involve blood + bone marrow
lymphoma
- tends to localize in lymph tissues
- often disseminated to other sites at diagnosis
plasma cell myeloma
- malignant transformation of B-cell plasma cells
- likes to form localized tumors in bony structures
lymphoid stem cells differentiate into…
1 NK cell
2 B cell
3 T cell
myeloid stem cells differentiate into…
1 neutrophil
2 monocyte
3 RBC
4 megakaryocyte
NK cell neoplasm
NK-cell leukemia/lymphoma
B cell neoplasm
B cell leukemia/lymphoma/myeloma
T cell neoplasm
T cell leukemia/lymphoma
Neutrophil neoplasm
granulocytic leukemia
Monocyte neoplasm
monocytic leukemia
RBC neoplasm
PV/erythroid leukemia
megakaryotic neoplasm
essential thrombocythemia/megakaryocytic leukemia
lymphoid vs myeloid neoplasms
LYMPH: leukemia, lymphoma, myeloma (B cells)
MYEL: leukemia, thrombocythemia (megakaryote)
___ of leukemia cells would also decide which types of blood cells would be affected
ASNAPLASIA
categories of hematologic neoplasms are based on ______ rather than its location in the body
based on cell type of the neoplasm
such as myeloid lineages (RBC, platelets, etc), or lymphoid lineage (NK, T, B cells)
types of myeloid neoplasms
1 Myoproliferative Disease
2 Myelodysplastic/Proliferative Disease
3 Myelodysplastic Syndrome
4 Acute Myeloid Leukemia (AML)
types of lymphoid neoplasms
1 B-cell neoplasm
2 T-cell + NK-cell neoplasm
3 Hodgkin disease
types of non-hodgkin lymphoma
lymphomas of B, T, NK cell origin
-includes large diverse group of malignancies
signs + symptoms of malignant WBC’s
- enlarged, nontender lymph nodes (lymphadenopathy) w lymphoma + some leukemias
- recurrent infections
- V high WBC count or presence of abnormal cell types
- malaise, weakness, unexplained fever, night sweats
- anorexia, weight loss
- hepatomegaly
- splenomegaly
clinical manifestations of hematologic neoplasms
- anemia
- thrombocytopenia
- leukopenia, neutropenia
tumor vs neoplasm
tumor refers to swelling or a lump like swollen state that would normally be associated with inflammation, whereas a neoplasm refers to any new growth, lesion, or ulcer that is abnormal
treatments for malignant WBC
- chemotherapy (primary)
- stem cell transplant (primary)
- radiation
- tissue-specific drug therapy
chemotherapy
- induce long-term remission
- removes malignant cells
- usual treatment includes MANY chemotherapy cycles
complete remission (CR)
return to normal hematopoiesis
-no detectable neoplastic cells
most chemotherapeutic agents induce ___
apoptosis
chemotherapy treatment phases
1 remission induction
2 post-remission or consolidation
3 remission maintenance
remission induction
- 1st phase of chemotherapy
- eliminate all detectable neoplastic cells + achieve CR
post-remission or consolidation
- 2nd phase of chemo after CR is attained
- eliminates an UNdetected cells (may have escaped initial induction-phase)
remission maintenance
- 3rd phase in chemo
- manages some neoplasms to prolong remission interval
prevention + mgmt of complications
1 bone marrow transplant (BMT)
2 peripheral stem cell transplantation
bone marrow transplant (BMT)
- used to manage certain leukemias
- manage bone marrow failure due to intense chemotherapy
- stem cells reintroduced to bone marrow via IV
peripheral stem cell transplantation
allows stem cells to be harvested fr circulating bloodstream
what may occur if bone marrow transplantation is NOT from a close match
graft-versus-host may occur
common types of Myeloproliferative Diseases
1 Chronic Myeloid Leukemia (granulocytes)
2 Polycythemia Vera (RBC)
3 Essential Thrombocytopenia
Chronic Myeloid Leukemia [CML]
avg age of onset
adult: 40-50 yrs
Chronic Myeloid Leukemia [CML]
usual clinical presentation
- ***high granulocyte count on the CBC
- spenomegaly
Chronic Myeloid Leukemia [CML] is characterized by _____
CML is characterized by MALIGNANT TUMORS that carry the Phladelphia chromosome [Ph+]
Ph+
philadelphia chromosome
- translocation of chromosomes 9 + 22 to create new fusion gene called bcr/abl
- causes myelogenous leukemia
-incr cell proliferation + reduce apoptosis
Chronic Myeloid Leukemia [CML]
treatments
- CML does NOT respond well to chemo (untreated patients have median survival of 2 yrs)
- ANTI-BCR/ABL THERAPY [IMATINIB]
imatinib
anti-bcr/abl therapy
-reduce number of leukemic cells w bcr/abl type to undetectable levels
Acute Myeloid Leukemia [AML]
onset
- abrupt onset of symptoms
- 80% of cases are adults
- median age 64 yrs
between AML and ALL, which has the worse prognosis?
AML (acute myeloid leukemia) has the worse likely course
-50% of kids + 30% of adults survive long-term
lymphoid neoplasms
- includes malignant transformations of B, T NK cells
- leukemia
- lymphoma
leukemia vs lymphoma
leukemia is present in blood or marrow
lymphoma is localized in lymphoid tissues
precursor cell neoplasms
characterized by cells that have arrested dvlpt in early blast stage
mature cell neoplasms
more differentiated than precursor cells;
often located in peripheral sites
Chronic Lymphoid Leukemia [CLL]
- most common leukemia in U.S. (30%)
- 95% are malignant B-CELL PRECURSORS
- –asymoptomatic, usually found by accident in routine blood exams
- 5% are aggressive T-CELL transformation
Chronic Lymphoid Leukemia [CLL] invades ______
lymphoid tissue + bone marrow
-disrupts function, enlarged + painless lymph nodes + spleen
Chronic Lymphoid Leukemia [CLL] infiltrates bone marrow and causes…
a reduction of RBC/platelet production
Acute lymphoblastic Leukemia/Lymphoma [ALL]
-primarily a children’s disorder (3-7 yrs, then middle age)
- causes severe bleeding
- hemathrosis
Plasma Cell Myeloma [Multiple Myeloma]
-malignant disorder of mature antibody-secreting B lymphocytes (aka plasma cells)
Plasma Cell Myeloma [Multiple Myeloma]
age of onset + location
-exclusively in adults 40 yrs or older, peak 65 yrs
- malignant plasma cells invade bones + form multiple tumors
- may also target lymph nodes, liver, spleen, kidney
Plasma Cell Myeloma produces….
- excessive identical monoclonal antibodies
- accumulates in bloodstream - bence jones protein/light-chain antibody fragments
- accumulates in blood + urine, kidney (damages kidney)
accumulation of monoclonal antibodies fr malignant plasma cells are detected by….
serum protein electrophoresis
when plasma cell myeloma accumulates in bone…
1 pathologic fractures are common
2 incr in Ca released in bloodstream fr bone destruction
Plasma Cell Myeloma [Multiple Myeloma]
diagnosis
based on: 1 monoclonal antibody peak 2 presence of bence jones protein 3 hypercalcemia 4 bone lesions
Bence Jones Protein
- light-chain antibody fragments
- produced by malignant plasma cell
- accumulates in blood + urine + kidney (damages kidney)
Hodgkin Disease
malignant disorder of lymph nodes
- 30% of malignant lymphoma
- occurs across lifespan (20-40 yrs)
- higher incidence + worse prognosis in males
- 5 yr survival rate
Hodgkin Disease
pathogenesis
- characterized by REED-STERNBERG CELLS
- originate fr B cells in germinal centers of lymph nodes (malignant but grow/spread in predictable way)
Hodgkin Disease usually metastasizes along ___
contiguous lymphatic pathways (predictable metastasis route)
_____ is frequently found in genome of transformed Reed-Sternberg (in Hodgkin disease)
Epstein-Barr virus
-usually present in single node or localized node chain
hodgkin disease is often asymptomatic in ___ stage
early stage
most common enlargement site of hodgkin
cervical nodes - lymph node enlargement above diaphragm
Hodgkin Disease
prognosis + treatment
stage dictates treatment modality
- localized tumors : radiation therapy (most common)
- disseminated disease : chemotherapy
Non Hodgkin
most arise from B, T, NK Cell
- no Reed-Sternberg cells
- spread early + unpredictably when compared to Hodgkin’s
Epstein Barr virus is known to cause
Burkitt lymphoma
Non Hodgkin lymphoma complications
2 serious oncologic emergencies:
1 superior vena cava obstruction
2 spinal cord compression
vocab
1 leukemia 2 lymphoma 3 plasma cell myeloma 4 myeloid 4 lymphoid stem cell
1 circulating tumors (blood + marrow)
2 localize in lymph tissue
3 malignant mature B cells, tumors localized in bony structures
4 stem cells that diff. into neutro, monocyte, RBC, megakaryote
most common form of leukemia
chronic lymphoid leukemia CLL
liver associated bleeding issue is due to a deficiency of ___
vitamin K
