White Blood Cell Disorders Flashcards by Rachel Eifert

What are lymphomas?

malignant proliferations of cells native to lymphoid tissue (lymphocytes, lymph precursors and derivatives)

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Lymphomas usually arise in lymphoid tissue and can spread to involve ____ tissue, ______, and _____.

solid tissue
marrow
blood

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What are the two main types of lymphomas?

Hodgkin

2. Non-Hodgkin

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What are leukemias?

malignant proliferations of cells native to the bone marrow, which often spill over into the blood

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True or False: Only lymphomas can involve lymphoid tissue.

False,

both leukemia and lymphoma can involve lymphoid tissue at any site

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Where do leukemias typically spread?

to solid tissues/organs (particularly the spleen and liver)

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Hodgkin Lymphoma is defined morphologically by the presence of _______ cells.

Reed-Sternberg

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True or False: Both forms of lymphoma present with fever, arise from a single lymph node, and are more common in young adults.

False, that is Hodgkin ONLY

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True or False: The cause of HL is unknown.

True, but EBV has been implicated in playing a role

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How is the Reed-Sternberg cell distinguished?

it is a LARGE neoplastic cell with mirror image nuclei and prominent nucleoli (Owl’s Eye)

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True or False: Large numbers of RS cells can be found in the HL involved node.

False, small numbers of RS cells are present in the involved node of HL (less than 2%)

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True or False: RS cells alone are adequate for the diagnosis of HL.

False, RS cells can be seen in non-neoplastic disorders like noninfectious mononucleosis

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How many types of HL are recognized? Do they present the same clinically?

5 types

different clinical presentations and histopathologic features

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______ is the term that refers to the assessment of the amount of tumor burden and its distribution in the body.

Staging

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The ______ virus is present in 70% of RS cells.

Epstein-Barr

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Choice of therapy is based on ______.

stage

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Describe a “low stage” disease.

localized lymph node involvement without systemic signs (fever, weight loss)
*better prognosis

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Describe “high stage” disease.

widespread disease, often with bone marrow involvement

*worse prognosis

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Treatment for HL consists of a combination of ______ and, now to a lesser extent, ______.

chemotherapy

radiotherapy

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Describe the “predictable spread” of HL.

lymph nodes
spleen and liver involvement (assessed with MRI)
Bone Marrow (decreased immunity, anemia, thrombocytopenia, etc)

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True or False: There is a risk for developing acute leukemia after treating HL with chemotherapy and radiotherapy.

True, because bone marrow toxicities of the drugs

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What are the Stages of HL?

I - tumor in one anatomic region or two on same side
II - tumor in more than two regions or two regions on opposite sides of the diaphragm
III - tumor on both sides of the diaphragm, not extending past nodes, spleen or Waldeyer’s ring
IV - tumor in bone marrow, lung, etc; any organ outside nodes, spleen or Waldeyer’s ring

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How are stages further divided?

A or B
A = absence
B = presence of systemic symptoms (fever, night sweats, and unexplained significant weight loss) = worse prognosis

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True or False: Both chemotherapy and radiotherapy are used to treat high and low level stages of HL.

False,
chemo or radio = low stage (localized)
chemo = high stage (widespread)

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Which stages are more likely to have "B" symptoms?

Stage III and IV

What is the 5 year survival rate for Stage I through Stage IIA of HL?

almost 100%

What is the 5 year survival rate for Stage IV HL?

50%

Non-Hodgkin Lymphomas (NHL) arise in _______ tissue and have the capacity to spread into __1___, ___2___, ___3__, and __4__.

lymphoid tissues (either nodes, or lymph tissue of solid organs) 1. other nodes 2. solid organs 3. bone marrow 4. blood

True or False: There is less morphological diversity in NHL than in HL.

False, there is more morpho diversity in NHL | *more than 2 dozen subtypes are recognized

How common are HL and NHL?

HL ~ 9k cases in 2015 | NHL ~71k cases in 2015

About _____ % of NHL are clonal neoplasms of _____.

85 | B-cell origin

If 85% of NHL are of B-cell origin, where do the other 15% originate?

T cells

How do NHL differ from Hodgkins?

NHL have: - multiple node involvement - more frequent extranodal spread and peripheral blood involvement - affect at all ages

All lymphoid neoplasms are considered to arise from a single transformed cell. Describe how this pertains to NHL.

B-Lymphocytes -normally have the capacity to differentiate into plasma cells as part of the immune response T-Lymphocytes -become activated as part of the normal immune response and secrete cytokines **Lymphoma = monoclonal expansion of lymphocytes that have been arrested at a particular stage = cells proliferate without normal regulatory mechanisms

How are NHL's classified (4 components)?

1. morphology 2. cell of origin 3. clinical features 4. genotype

NHL shows systemic symptoms in ____% of patients.

True or False: NHL is associated with painful lymph node enlargement.

False, the nodes enlarge but it is PAINLESS

There is less correlation between stage and prognosis with NHL (unlike HL). What are better determinants of prognosis?

1. cell type (aka: NHL Subtype = grading) | 2. tumor proliferative index

NHL can involve the ______, _____, and _____.

GI Tract Bones CNS

Describe the Stages of NHL.

I - single node or extralymphatic organ/site II - two or more nodes on same side of diaphragm or with involvement of extralymphatic organ III - lymph nodes on both sides of diaphragm (could include spleen) IV - multiple or disseminated foci of one or more organs/tissues, or tissues with/without lymphatic involvement **However: remember NHL prognosis is more based on grading than staging (HL is opposite)

True or False: Bone marrow involvement is more common in HL than in NHL.

False, more common in NHL

Which lymphoma type can be treated with bone marrow transplants?

highly resistant NHL, transplant with high dose chemo

NHL can be classified by its growth pattern: _____ or _____. Which has a worse prognosis?

Nodular or Diffuse | nodular is better, diffuse is bad

NHL can be classified based on _____: small or large. Which gives a better prognosis?

cell size | small is better than large

How are leukemias classified?

1. Acute | 2. Chronic

True or False: Leukemias arise in the bone marrow and often spill over into the blood or other organs.

True

What is the difference between acute and chronic leukemia?

Acute: RAPID onset with BLASTS in the blood Chronic: INDOLENT onset with more MATURE cells

True or False: In both acute and chronic leukemia, myeloid and lymphoid cells are affected.

True

What are the four types of leukemia?

``` Lymphoblastic: ALL CLL Myelogenous: AML CML ```

What is the most frequent type of leukemia in children less than 15 years old? What are other characteristics of this leukemia?

Acute Lymphoblastic Leukemia (ALL) - accounts for about 40% of acute leukemias - clonal growth of PRIMITIVE LYMPHOID cell - both B and T cell types - increased WBC and decreased platelets - OFTEN involves the CNS - enlargement of nodes, liver, and spleen

Which type of ALL has the best prognosis?

pre-B type | age 2-10 group in particular

What are the subtypes of ALL?

``` early B precursor pre-B mature B T cell hyperdiploidy (more than 50 chromosomes per cell) ```

What is the proliferating cell type in AML?

primitive myeloid cell

True or False: AML is more common in adults.

True, median age is 50 | **differentiation includes more mature myeloid cells with granules (myeloperoxidase)**

When present, the cytoplasmic inclusions called ______ are diagnostic for AML.

Auer Rods

AML cells will sometime proliferate the soft tissue, including the ______, and produce ________ sarcoma.

granulocytic

Is remission common in AML after chemotherapy?

yes, 70%

The proliferating cell in CLL is an ________ _____ lymphocyte that appears mature.

immunologically incompetent

More than 95% of Chronic Lymyphocytic Leukemias are for which cell type? Most commonly expressing which surface immunoglobulin?

B Lymphocyte | surface IgM with Kappa light chain restriction

Which leukemia is most common in adults over 60 years old?

CLL | **accounts for 2/3 of chronic leukemias**

What is the male to female ratio for CLL?

2:1 (male to female)

In CLL there is an increased white cell count and increased expression of ______.

BCL2

The median survival rate for CLL is 4-6 years as the tumor cells suppress normal ______ actions and cause ________ in most patients.

B cell | hypogammaglobulinemia

True or False: All cases of CLL eventually terminate into a higher grade process, such as acute leukemia or high grade lymphoma.

True

What is the proliferating cell in Chronic Myelogenous Leukemia?

immature hematopoietic cell

CML is associated with an increase in white blood cell count with _______ and/or _______.

eosinophilia | basophilia

Which chromosome abnormality occurs in proliferating cells of CML?

Philadelphia Chromosome (9 and 22) which causes fusion of the BCR-ABL tyrosine kinase genes that mimic the effects of growth factor activation (driving proliferation)

What age range is affected by CML and CLL?

``` CML = 25-60 CLL = over 60 (later leukemia) ```

What anatomic alteration is almost always present with CML?

enlargement of the spleen (splenomegaly) due to proliferation of abnormal cells

What is "blast crisis" and with which form of leukemia is it related?

-a relative increase in immature cells in peripheral blood/bone marrow and decreased response to treatment, in the terminal phase of CML

From what two events do the clinical features of leukemia arise?

1. impairment of marrow function as abnormal cells suppress growth of normal cells 2. infiltration of body organs due to proliferation of the abnormal cells

How does anemia manifest?

pallor, weakness, and fatigue

Thrombocytopenia causes _____ and _____.

bleeding | bruising

Why do infections occur in conjunction with leukemia?

decreased production of mature granulocytes and production of non-functional granulocytes and/or lymphocytes

Fever with leukemias can be due to infection of increased _______.

metabolism of proliferating cells

True or False: Chest pain or obstruction of vascular and lymphatic channels can result from leukemia.

False, abdominal pain not chest pain

Infiltration of the gingiva is a feature commonly associated with ______ leukemias.

acute myelo-monocytic

What are "plasma cell disorders?"

- clonal expansion of immunoglobulin-secreting cells that result in increased serum monoclonal protein - increases in serum monoclonal protein (M component) which may have adverse effects on renal and neurologic function

What is Multiple Myeloma?

- a plasma cell disorder | - proliferating cell is a PLASMA CELL that produces one immunoglobulin type (heavy or light chain)

What are the three most common immunoglobulins that proliferate in multiple myeloma?

IgG (60%) IgA (20-25%) kappa or lamda light chain (the remaining 15%)

Multiple Myeloma is a disease that affects those of ________ age.

late middle age (to elderly)...average around 70 years

How many cases of Multiple Myeloma are predicted for 2016?

30,000

When only light chains are produced, patients can excrete the _____ molecular weight chains in the urine. This is referred to as _______.

low | Bence Jones proteinuria

_______ bone lesions characterize myeloma.

multifocal destructive (punched out, honey-comb areas of bone)

Proteinaceous casts may form in the ______ due to multiple myeloma.

kidneys

What are the blood and marrow features associated with MM?

- RBC stacking (rouleaux formation) | - marrow plasma cell infiltrates in single cells and sheets

True or False: The survival time of the typical form of Multiple Myeloma is 10 years.

False, 4-6 years typically

How is multiple myeloma treated?

- chemotherapy (thalidomide analogs; anti-resorptives) - radiotherapy (palliation for bone pain) - bone marrow transplant