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Hematopathology IV Flashcards

Oncogenic Diseases of Blood Cells (56 cards)

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1
Q

What do lymphomas typically appear as?

A

Tumor masses in lymph nodes or lymphoid aggregates in organs

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2
Q

All lymphoid neoplasms tend to spread to

A

Lymph nodes and other tissues, spleen, liver, bone marrow

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3
Q

Lymhomas can spill over into blood giving

A

a leukemic picture (and leukemias can form masses)

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4
Q

Most T and B lymphomas composed of cells that have

A

stopped at a particular stage of differentiation

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5
Q

Diagnosis of T and B lymphomas depends heavily on

A

maturation markers

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6
Q

What origin are most common lymphomas in adults?

A

B cell origin

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7
Q

Are B or T cells more genetically stable and therefore have fewer lymphomas?

A

T cells

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8
Q

B cell origin lymphomas: Common chromosomal translocations involve what gene loci?

A

Ig

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9
Q

B cell origin lymphomas are dervied from

A

Follicle where cells usually undergo regulated somatic hypermutation that places cells at risk of mutation

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10
Q

Non-Hodgkin lymphomas are often widely disseminated at time of diagnosis, requiring what?

A

Systemic therapy

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11
Q

Hodgkin lymphomas often present at a

A

Single site and spread from node to node in a chain

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12
Q

Hodgkin lymphomas may be cured with

A

Local therapy early in course of disease

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13
Q

Are lymphoid neoplasms clonal?

A

Yes

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14
Q

Lymphoid neoplasms disrupt

A

normal immunity
- Immunodeficiencies, autoimmunity accompanies them

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15
Q

Precursor B/T cell lymphoblastic leukemia/lymphoma both rapidly progress to a leukemic phase as

A

acute lymphoblastic leukemia (ALL)

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16
Q

Pre-B lymphoblastic leukemia/lymphoma occurs in who?

A

Young kids

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17
Q

Pre-T lymphoblastic leukemia/lymphoma mainly occurs in who?

A

Teens

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18
Q

Describe the onset of pr B/T cell lymphoblastic leukemia/lymphoma

A

Abrupt. Symptoms related to marrow failure, bone pain, hepatosplenomegaly, enlarged lymph nodes

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19
Q

Pre-B lymphoblastic tumors arise in

A

bone-marrow and pre-T lymphoblastic tumors arise in thymus as mediastinal mass

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20
Q

Describe symptoms of small lymphocytic lymphoma/chronic lymphocytic leukemia

A

Can be asymptomatic, but symptoms can reflect marrow failure, enlarged organs, bone pain

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21
Q

Describe Small lymphocytic lymphoma/chronic lymphocytic leukemia

A
  • B cell
  • Same disease, differing in extent of peripheral blood content
  • Most are diagnosed at CLL stage, common in adults, course is variable
  • Some die with disease while in other cases, a new, more aggressive clone emerges dropping survival duration
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22
Q

Describe follicular lymphomas

A
  • B cell
  • Older patients
  • Painless lymphadenopathy, often generalized including bone
  • Uncommon in extranodal sites, uncommon to be leukemic
  • long natural history, indolent and generally incurable
  • About 40% develop a new, more aggressive clone giving rise to diffuse lymphoma and worse prognosis
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23
Q

In follicular lymphomas, most have a

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A

t(14:18) translocation with overexpression of BCL-2 gene (anti-apoptosis)

24
Q

Describe Large B cell lymphomas

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A
  • Aggressive
    0 EBV implicated in those occurring in immunocompromised
  • Most occur in older adults (but wide range)
  • fatal if not treated
25
Large B cell lymphomas typically present with
rapidly enlarging single mass at nodal or extranodal site Liver spleen and bone marrow not usually invovled at time of diagnosis and leukemic component is rate
26
Treatment for large B cell lymphomas
Combination chemotherapy (puts most into complete remission)
27
Describe Endemic Burkitt lymphoma
- Parts of Africa, strongly associated with EBV infection - Translocation of MYC gene - Malaria, other infections reduce immunocompetence allowing EBV to cause B cell proliferation - Children/young adults. Maxilla or mandible is most common presentation in African from
28
Describe Non-endemic Burkitt lymphoma
- MYC translocation, but only 20% have EBV - Prresents as an abdominal tumor
29
Both types of burkitt lymphomas are
High grade, aggressive tumors. Need aggressive chemo
30
Multiple myeloma originates from
Clone of B cells that differentiated into plasma cells and secrete single complete Ig or Ig fragment into blood
31
Describe multiple myeloma
- Proliferation of neoplastic plasma cells in bone marrow - Multifocal lytic lesions in skeleton, mainly in older adults - IgG or IgA is usual M component (kappa and lambda light chains get into urine as Bence-Jones protein) - No cure, median survival 4-6yrs
32
Symptoms of Multiple myeloma
Bone pain, fractures, hypercalcemia, myelophthisic anemia, infections, amyloidosis
33
How is Hodgkin lymphoma diagnoses histologically?
Identifying Reed-Sternberg cells
34
Stage 1 Hodgkin lymphoma
Single lymph node chain
35
Stage II Hodgkin lymphoma
More than one chain on same side of diaphragm
36
Stage III Hodgkin lymphoma
Both sides of diaphragm
37
Stage IV Hodgkin lymphoma
Widely disseminated disease
38
Younger patients with more favorable histologic subtypes of Hodgkin Lymphoma tend to present in what stages?
Stages I and II (usually asymptomatic, enlarged cervical lymph node)
39
Hodkin lymphoma stages III/IV symptoms
Fever, weight loss, anemia, night sweats
40
what are myeloproliferatiive neoplasms?
- Chronic myeloid leukemia - Polycythemia vera - Primary myelofibrosis - Essential thrombocythemia - Chronic eosinophilic leukemia, not otherwise specified - Matocytosis - Myeloproliferative neoplasm, unclassifiable
41
What are myelodysplastic/myeloproliferative neoplasms?
- Chronic myelomonocytic leukemia - Atypical chronic myeloid leukemia, bur/abl negative - Juvenile myelomonocytic leukemia - Myelodysplastic/myeloproliferative neoplasm, unclassifiable
42
Describe myelodysplastic syndrome (MDS)
- Transformed stem cell differentiates into a clone of abnormal RBCs, granulocytes and platelets - Ineffective hematopoiesis: cytopenias - Many cases eventually progress to AML
43
How is myelodysplastic syndromes classified?
- Number of lineages displaying dysplasia - % blasts in peripheral blood/bone marrow - Cytogenetic aberrations identified
44
Describe myeloproliferative neoplasms
- Proliferation of progenitors that retain capacity to terminally differentiate - Increase in mature blood elements
45
Describe chronic myeloid leukemia
- All cases have the t(9;22) Philadelphia chromosome - BCR/ABL balanced reciprocol translocation - Pluripotent stem cells produce a number of myeloid cells (neutrophils, myelocytes, basophils in high numbers) - Slowly progresses- chronic - Progression to accelerated phase and blast crisis - Clonal evolution
46
Most acute myeloid leukemias harbor mutations that interfere with
differentiation of myeloid cells
47
Most acute myeloid leukemias have >20%
myeloblasts in peripheral blood or BM
48
Polycythemia denotes an
increase in RBC with increase in Hb
49
What is relative polycythemia due to?
Decreased plasma volume as occurs when dehydrated
50
What is absolute polycythemia?
Increase in total RBC mass
51
Primary form of absolute polycythemia is
Polycythemia vera, a neoplastic proliferation of progenitor cells
52
Secondary form of absolute polycythemia is-
a response to increased erythropoietin due to a high altitude, lung diseases, cyanotic heart disease, RCC synthesizing excess erythropoietin
53
What is Langerhans Cell histiocytosis?
Proliferation of Langerhans cells (immature dendritic cells) - APC MHC Class II antigens; common in skin, mucosa
54
Describe Chronic localized LCH
- Affects adolescents, young adults - One or a few sites, usually in bone that can cause symptoms - Indolent, lesions may heal spontaneously or require surgery or radiation - Each focus of disease is often called an eosinophilic granuloma
55
Describe Unisystem LCH (Eosinophilic granuloma, obsolete)
- Affects older children, less aggressive - Unifocal or multifocal erosive expansion of LC - Bones (erosive, lytic lesions), skin, lungs, GI tract - Spontaneous regression vs. require intervention/treatment
56
Multistem/Disseminated LCH (Letterer-Siwe disease, obsolete)
- Infants < 2 yrs - Infiltrates skin (seborrheic keratosis like rash) and many organs (liver, spleen) and osteolytic lesions - Fatal if untreated - With chemo, 50% 5 yr survival

Systems 1 (32 decks)

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