WBC disorders Flashcards by asw asw

Hodgkins lymphoma is a lymphoma of a ______________

distinctive cell type

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which specific cells are the malignant cells of hodgkins lymphoma?

Reed-Sternberg cells

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what are the pathological characteristics of hodgkins lymphoma?

Arises in a single lymph node or spreads contiguously to involve a chain of nodes

Highly curable in most cases with chemotherapy and/or radiotherapy

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what are the CLINICAL signs/symptoms of Hodgkins lymphoma? (theres 3 points)

1) Bimodal age distribution: 20-30 years old and >50 years old
2) Painless lymphadenopathy (often cervical, supraclavicular, mediastinal)
3) Splenomegaly (detected by MRI)

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how are cases of Hodgkins lymphoma diagnosed?

Lymph node biopsy necessary for diagnosis

Diagnosis requires identification of Reed-Sternberg cells in appropriate background

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there are _____ different types of Hodgkins lymphoma. How are they recognized?

Five different types

each is recognized with their own clinical presentations and histopathologic features

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what are the characteristics of Reed-Sternberg (RS) Cells?

Large cell with mirror-image nuclei and prominent nucleoli
derived from B lymphocytes
The malignant cell of Hodgkin lymphoma, but comprises only 2% of cells in the involved lymph node

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what virus is often found in reed-sternberg cells involved in Hodgkins lymphoma?

Epstein-Barr virus (70%)

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____________ is used to determine treatment and prognosis for patients with Hodgkins lymphoma

staging

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what are the two stages of Hodgkins lymphoma?

Low stage:
- Localized involvement

High stage:
- Wide spread disease with distant or bone marrow involvement

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what is the definition of stage 1 hodgkins lymphoma? what about stage 2?

Stage 1: Tumor in one anatomic region or two contiguous anatomic regions on the same side of the diaphragm

Stage 2: Tumor in more than two anatomic regions or two non-contiguous regions on the same side of the diaphragm

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how do stages 3 and 4 of hodgkins lymphoma differ?

stage 3:
- Tumor on both sides of the diaphragm not extending beyond lymph nodes, spleen or Waldeyer’s ring (tonsils)

stage 4:
- Tumor in bone marrow, lung, etc.- any organ site outside of the lymph nodes, spleen or Waldeyer’s ring

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what are the “B” signs/symptoms of Hodgkins lympoma?

Fever, night sweats, and significant unexplained weight loss

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the treatment for Hodgkins lymphoma is based primarily on ___________

the stage of the disease

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what are the treatments for low stage (localized) hodgkins lymphoma? what about high stage?

Low stage: Chemotherapy and radiotherapy

high stage: Chemotherapy

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T/F: patients with hodgkins lymphoma are at a high risk for development of secondary treatment-related acute leukemia

FALSE

they are at low risk

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hodgkins lymphoma Patients without _____________ have better prognosis

“B” signs/symptoms

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what is the survival rate for stage 1 & 2 hodgkins lymphoma? how about later stages?

5-year survival - Stage I and IIA - almost 100%

5-year survival rate in Stage IV is 50%

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what are the characteristics of Non-Hodgkin Lymphoma (NHL)?

Neoplastic lymphocytes originating in lymph nodes or extranodal lymphoid tissue
Most (85%) are of B cell origin
Most of remainder are of T cell origin
Incidence rises steadily after age 40

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how many different types of NHL are there?

Over 2 dozen types

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list the clinical signs of NHL

Painless lymph node enlargement

Systemic symptoms in 30% of patients

Frequent immune abnormalities

Splenomegaly

May involve GI tract, bones, central nervous system

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how are different types of NHL classified?

Growth pattern
- Nodular vs diffuse

Cell size
- Small vs large

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is nodular or diffuse Non-Hodgkins lymphoma better? how about small vs large cell size?

Nodular disease better than diffuse

Small cell disease better than large

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how is the prognosis for NHL determined?

Prognosis often based more on the sub-type of lymphoma than stage
(exception to the general rule, “grading” or subtyping is critical)

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what are the treatments for non-hodgkins lymphoma?

Chemotherapy Possible radiation therapy Bone marrow transplantation

what are the characteristics of acute leukemia?

Rapid onset with blasts in the blood Myeloid and lymphoid cells affected

what are the characteristics of chronic leukemia?

Indolent onset and tends to involve more mature cells Myeloid and lymphoid cells affected

___________ are malignant neoplasms of hematopoietic tissue that arise in the bone marrow

Leukemias

what are the 4 basic classes of leukemia?

acute lymphoblastic leukemia (ALL) chronic lymphocytic leukemia (CLL) acute myelogenous leukemia (AML) chronic myelogenous leukemia (CML)

what are the general characteristics of acute lymphoblastic leukemia (ALL)?

- Clonal growth of primitive lymphoid cell - Both B and T cell types exist - often involves the central nervous system - Increased white blood cell count often accompanied by thrombocytopenia

in what population is acute lymphoblastic leukemia most common?

Most common in children ***most frequent cancer in those < 15 yrs of age****

the Prognosis of ALL is related to ___________

cytogenetics **hyperdiploidy (>50 chromosomes per leukemic cell)** the subtype with best prognosis (most are children)

what population usually has a poor prognosis for acute lymphoblastic leukemia? who usually has a good prognosis?

Children have good prognosis Adults often have bad prognosis

list the general characteristics of Acute Myelogenous Leukemia (AML)

- Clonal proliferation of primitive myeloid cell - Increased WBC count often accompanied by anemia and thrombocytopenia - More common in adults

during acute myelogenous leukemia, granules of ______________ are present in the cytoplasm of more mature myeloid cells

Myeloperoxidase

the prognosis for AML is influenced by __________

cytogenetics | the chromosome mutation is the most important factor in prognosis

list the general characteristics of Chronic Myelogenous Leukemia (CML)

- Clonal proliferation of mature lymphocyte, typically a B lymphocyte - The B cells are immunologically incompetent - Monoclonal expression of surface immunoglobulin

CML is most common in what population?

adults over 60 years old

during CML, there is an Increased white cell count with ___________

lymphocytosis

what are the pathogenic characteristics of CML?

- Increased white cell count with lymphocytosis - splenomegaly and lymphadenopathy - Anemia and thrombocytopenia eventually develop as disease progresses

All CML cases eventually terminate into a higher grade process - either __________ or _________

acute leukemia or high grade lymphoma

as CML progresses, __________ may develop (anemia and thrombocytopenia) as the marrow is overrun by leukemic cells

cytopenias

what is the proliferating cell of CML?

an immature hematopoietic cell | a stem cell from which all other hematopoietic cells arise

During CML, a specific chromosomal abnormality in the ________________, occurs in all the proliferating cells

Philadelphia chromosome t(9;22)

The chromosomal abnormality of t(9;22) results in fusion of the __________ genes

BCR-ABL

what molecule is targeted during chemotherapy for CML treatment?

Targeted chemotherapy blocks the bcr-abl tyrosine kinase - induces remission in most patients

what treatments are available for relapsed/resistant CML?

Bone marrow transplant

what are the 3 common characteristics of all leukemias?

- Abnormal cells suppress growth of normal cells - Infiltration of body organs by abnormal cells - Immune dysfunction

Multiple Myeloma is the result of ___________ proliferations

plasma cell proliferations

what are the general pathological characteristics of multiple myeloma?

- A clonal proliferation of monoclonal plasma cells - Monoclonal heavy and or light chain production - A disease of late middle age to elderly

which immunoglobulin is most commonly produced during multiple myeloma?

IgG

Plasma cell disorders result from clonal expansion of ______________-secreting cells

immunoglobulin

what are the Clinical/Laboratory Findings for multiple myeloma?

- Multiple lytic bone lesions, hypercalcemia - Bone marrow infiltration by plasma cells - ↑↑ serum monoclonal protein, but normal immunoglobulins are suppressed - Renal failure - Infections

Bence-Jones proteinuria is caused by what kind of immunoglobulin?

light chain Ig's

what are the Blood and Marrow Features of patients with Multiple myeloma?

- Circulating plasma cells in blood are uncommon (they are mostly found in the marrow) - RBC show rouleaux formation (stacks of clumped cells) - Marrow plasma cell infiltrates in single cells and sheets

what are the prognosis for the indolent and typical forms of multiple myeloma?

Indolent form: survival for years (don't need treatment) Typical form: median survival – 4-6 years

what are the treatments available for multiple myeloma?

Chemotherapy – recent success with thalidomide analogs, anti-resorptive tx Radiotherapy (palliation – for bone pain) Bone marrow transplant

patients with multiple myeloma often present with what clinical signs?

Patients often present with bone pain, hypercalcemia, and renal disease ``` The "CRAB" symptoms C- calcium (elevated) R- renal failure A- anemia B- Bone pain/lesions ```

what is the group with the best prognosis for Acute Lymphoblastic Leukemia?

children aged 2-10 with pre-B cell type

Enlargement of lymph nodes, liver and spleen is more common in ALL than _______

AML acute myelogenous leukemia

In cases of AML, Cytoplasmic inclusions called _________, when present, are diagnostic

Auer rods

the ___________ of AML cells is the most predictive factor for prognosis

The karyotype is most predictive of prognosis

what is responsible for granulocytic sarcomas?

AML lesional cells proliferate into soft tissue (including the gingivae)

T/F: remission of AML is usually transient, not permanent

true

More than 95% of CLL are of what type?

B cell type

what immunoglobulin do most CLL cells produce?

IgM kappa surface immunoglobulin

This type of leukemia accounts for about 2/3 of chronic leukemias

Chronic Lymphocytic Leukemia

____ accounts for about 1/3 of chronic leukemias

CML

what is blast crisis?

- occurs when treating terminal phase CML | - its a relative increase in immature cells in peripheral blood and bone marrow, and decreased response to treatment

This stage of CML is equivalent to an acute leukemia

terminal phase with blast crisis