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General Pathology > LN and WBC Disorders > Flashcards

LN and WBC Disorders Flashcards

1
Q

Another name for white blood cells

A

leukocytes

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2
Q

how many main compartments of the body do leukocytes occupy?

A

4

(Disorders can begin in any of these locations and then spread to the others)

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3
Q

what are the 4 main compartments of the body that leukocytes occupy? What is the relationship of each of these compartments to the WBC?

A

-bone marrow –> production of WBCs

-bloodstream –> transport

-lymph nodes –> immune activation

-site of infection or immune stimulation –> can be within any organ or soft tissue (what you see clinically)

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4
Q

T/F PNMs only refer to neutrophils

A

false –> refer to all granulocytes

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5
Q

what is leukopenia?

A

decreased serum level of leukocytes

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6
Q

what is leukocytosis?

A

elevated serum levels of leukocytes, mostly neutrophils –> 15-20,000/mm3

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7
Q

what is the normal expected value for WBCs in a CBC?

A

4 - 10,000/mm3 (or µl)

(elevates to 15-20,000/mm3)

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8
Q

what is the main cause of neutrophilic leukocytosis? (2)

A

-bacterial infections
-tissue necrosis (burns, myocardial infarctions)

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9
Q

what is the most common type of leukocytosis?

A

neutrophilic leukocytosis

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10
Q

what is the main cause of lymphocytotic leukocytosis? (2)

A

-chronic infections
-some viral infections

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11
Q

what is the main cause of monocytotic leukocytosis? (1)

A

-chronic infections

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12
Q

what is the main cause of eosinophilic leukocytosis? (3)

A

-allergies (asthma, hay fever)
-parasitic infections
-drug reactions

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13
Q

what is seen during a normal Lymph node (LN) evaluation?

A

LNs normally small (<0.5cm) and nonpalpable

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14
Q

what is seen during a Lymph node (LN) evaluation where Lymphadenopathy (LAD) is present??

A

firm, enlarged LN (>1cm)

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15
Q

where is painful LAD normally seen?

A

in the LN that is draining a region of infection (acute lymphadenitis)

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16
Q

where is non-painful LAD normally seen?

A

with chronic inflammation (chronic lymphadenitis), metastatic cancer or lymphoma

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17
Q

most cases of Lymphadenopathy (LAD) are ____ and ____, particularly in children

A

self-limited and benign (i.e. not malignancy/ cancer)

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18
Q

does localized or generalized Lymphadenopathy (LAD) have more of a chance of underlying systemic disease?

A

generalized

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19
Q

describe what is meant by “significance of duration” when it comes to Lymphadenopathy (LAD)

A

-if <2 weeks or >1 year without change in size = unlikely to be a tumor (neoplasm)
-if >6 weeks and not better by 12 weeks = risk for cancer

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20
Q

what does an LAD workup consist of? (3)

A

serology (blood tests), imaging (ultrasound, CT) and possibly biopsy

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21
Q

what is the MIAMI mnemonic for different etiologies of LAD?

A

-Malignancies

-Infections

-Autoimmune disorders

-Miscellaneous/unusual conditions

-Iatrogenic causes

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22
Q

what are symptoms of a Malignancy-caused LAD?

A
  • fever
  • drenching night sweats
  • unexplained weight loss of greater than 10% of body weight
  • in supraclavicular LAD: intraabdominal malignancy (50%)
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23
Q

what are symptoms of an Infection-caused LAD?

A

fever, chills, fatigue, and malaise

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24
Q

what are symptoms of a Miscellaneous/unusual conditions-caused LAD?

A

other specific findings of each condition

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25
Q

what are symptoms of an Autoimmune disorder-caused LAD?

A

arthralgias, muscle weakness, and rash

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26
Q

what are symptoms of an Iatrogenic-caused LAD?

A

history of new medications

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27
Q

what LNs drain the oral cavity?

A

submandibular nodes

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28
Q

what is neutropenia?

A

decreased neutrophils in blood

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29
Q

what is Agranulocytosis?

A

decreased granulocytes (neutrophils, basophils an eosinophils) in blood

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30
Q

what are the major causes of neutropenia and Agranulocytosis? (2)

A

-Cancer chemotherapy
-Reaction to drugs

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31
Q

what is the most common sequellae of neutropenia and Agranulocytosis?

A

infection (particularly when below 500 cells/μl)

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32
Q

what are the major pathogenesises of neutropenia and Agranulocytosis?

A

-Decreased production in bone marrow
-Increased destruction of peripheral cells leads to hypercellular marrow

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33
Q

what are symptoms of neutropenia and Agranulocytosis?

A

Malaise, fever, chills, weakness, ulceration (oral, often gingival)- deep, punched out

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34
Q
A
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35
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36
Q
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37
Q

what can cause decreased bone marrow and therefore decreased production of neutrophils and/or granulocytes? (3)

A

-Chemo tx can cause a transient marrow hypoplasia decreasing neutrophil production

-Pts with aplastic anemia have chronic marrow hypoplasia

-leukemia causes replacement of the normal marrow

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38
Q

what is the treatment for Neutropenia/Agranulocytosis? (3)

A

-Remove the offending agent
-Control infections (antibiotics, antifungals etc.)
-Give granulocyte colony-stimulating factor (G-CSF) to stimulate granulocyte production

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38
Q

what can cause the increased destruction of peripheral cells that leads to hypercellular marrow? (3)

A

-immune-mediated injury (drugs)
-overwhelming infection (using up peripheral cells)
-splenomegaly (can accelerate removal of granulocytes)

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39
Q

how are WBC Neoplasms classified?

A

based on morphologic and molecular criteria (lineage-specific protein markers and genetic changes)

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40
Q

what are the broad categories of WBC Neoplasms based on?

A

origin and differentiation

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41
Q

what are the 3 major broad categories of WBC Neoplasms?

A

Lymphoid neoplasms
-Myeloid neoplasms
-Histiocytic neoplasms (Langerhans cell histiocytosis)

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42
Q

what are 2 etiologies for lymphoid neoplasms? which is more common

A

-increased risk for translocations and transformation in B cells (more common)

-T cells are genomically stable (uncommon cause of lymphomas)

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43
Q

Lymphoid neoplasms can result due to increased risk for translocations and transformation in B cells because in germinal centers they undergo what 2 things?

A

-somatic hypermutation
-class switching

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44
Q

why do B cells undergo somatic hypermutation in the germinal center?

A

to increase antibody affinity

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45
Q

why do B cells undergo class switching in the germinal center?

A

to produce multiple antibody types
(i.e. from IgM to IgG, IgA, IgE) to the same antigen

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46
Q

Know what germinal centers look like in lymph nodes

A

germinal centers are the large circles in the image

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47
Q

what are the parts of the LN germinal center?

A

-Dark Zone
-Light Zone
-Mantle Zone

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48
Q

T/F lymphomas can develop at any step during the normal maturation of B-cells

A

true

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49
Q

what occurs in the LN’s Dark Zone of the germinal center?

A

initial antigen stimulation

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50
Q

what occurs in the LN’s light Zone of the germinal center?

A

apoptosis and class switching

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51
Q

Group of hematologic malignancies characterized by tumor cells that originate in the bone marrow and spill over into the blood

A

Leukemia

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52
Q

Tumor masses in lymph nodes or other tissues

A

Lymphoma

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53
Q

All lymphoid neoplasms can spread to ______ and _____

A

lymph nodes and other tissues

(liver, spleen, bone marrow and peripheral blood)

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54
Q

Because of the overlap in clinical behavior and location, classification of Lymphoid Neoplasms focuses on what?

A

the morphology (shape, size) and the molecular characteristics (surface markers) of the tumor cells

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55
Q

______ tumors come from cells arrested at or derived from a specific stage of normal lymphocyte differentiation.

A

B and T cell

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56
Q

T/F Lymphoid Neoplasms cells change morphology and surface markers as they progress through each stage of differentiation

A

true

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57
Q

what are the B-cell neoplasms that can occur in the bone marrow before the cell matures in the lymph node? (4)

A

-Precursor B lymphoblastic lymphoma/leukemia
-small lymphocytic lymphoma
-chronic lymphocytic leukemia
-multiple myeloma

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58
Q

what are the B-cell neoplasms that can occur in the mantle zone of the LN? (1)

A

mantle cell lymphoma

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59
Q

what are the B-cell neoplasms that can occur in the germinal center of the LN? (4)

A

-Follicular lymphoma
-Burkitt lymphoma
-Diffuse large B-cell lymphoma
-Hodgkin’s lymphoma

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60
Q

what are the B-cell neoplasms that can occur in the marginal zone (post-germinal center) of the LN? (4)

A

-Diffuse large B-cell lymphoma
-marginal zone lymphoma
-small lymphocytic lymphoma
-chronic lymphocytic leukemia

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61
Q

what T-cell neoplasm occurs in the thymus before the T-cell reaches the LN?

A

Precursor T lymphoblastic lymphoma/leukemia

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62
Q

what T-cell neoplasm occurs in the the LN?

A

Peripheral T cell lymphomas

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63
Q

what are the WHO classification criteria for Lymphoid Neoplasms? (4)

A

-Morphology (H&E appearance)

-Cell origin (immunophenotyping by IHC and/or

flow cytometry)

-Clinical features

-Genotype (karyotype, presence of viral genomes)

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64
Q

______ and ______ account for the largest proportion of cancers in children by tumor type

A
  • Leukemia
  • lymphoma
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65
Q

Leukemias can have diffuse infiltration into what organs? what does this cause?

A

lymph nodes, spleen, liver and gingiva causing general enlargement

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66
Q

in general, what are Leukemias derived from?

A

a single transformed cell exhibiting clonal growth

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67
Q

T/F Typically the clonal population for a leukemia has different surface markers

A

false

Typically, the clonal cell population all have the same surface markers

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68
Q

what can be an etiology for Acute Leukemia? (4)

A

-Ionizing radiation (atomic bomb)

-Toxins (benzene and toluene)

-Antineoplastic chemotherapeutic drugs

(procarbazine, melphalan and other alkylating agents and etoposide)

-chromosomal abnormalities

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69
Q

what type of acute leukemia does Ionizing radiation most often lead to?

A

a myeloid leukemia

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70
Q

in the pathophysiology of Acute Leukemia there is myelophthisic anemia, which is the replacement of normal hematopoietic cells (myeloblasts, erythroblasts, and megakaryocytes) in bone marrow, causing what? (3)

A

-Neutropenia
-Thrombocytopenia
-anemia

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71
Q

when acute leukemia causes Neutropenia, what can this lead to?

A

bacterial, viral and fungal infections (oral ulcers, herpes, candida)

72
Q

when acute leukemia causes Thrombocytopenia, what can this lead to?

A

bleeding and petechiae (gingival bleeding and palatal petechiae)

73
Q

when acute leukemia causes Myelophthisic anemia, what can this lead to?

A

hypoxia

74
Q

Acute Leukemia patients often present with signs and symptoms related to pathophysiology?

A

myelophthisic anemia

75
Q

what are general symptoms of acute leukemia? (4)

A

-Fatigue, shortness of breath (SOB), pallor (decreased RBC’s)
-Easy bruising (decreased platelets)
-Infection-bacterial and fungal mostly (due to decreased/dysfunctional WBC’s)
-extramedullary hematopoiesis

76
Q

what is the most common cancer in children?

A

Acute Lymphoblastic Leukemia (ALL)

77
Q

what is Acute Lymphoblastic Leukemia (ALL) derived from?

A

immature B (pre-B) or T (pre-T) cells called lymphoblasts

78
Q

90% of Acute Lymphoblastic Leukemia (ALL) patients show ____ and ____, creating an aberrant ____

A

show hyperdiploidy and a translocation creating an aberrant transcription factor

79
Q

what is hyperdiploidy?

A

49-65 chromosomes/cell

80
Q

What is a neoplasm of mature circulating lymphocytes (high WBC count), and can be either lymphocytic or myeloid

A

Chronic leukemia

81
Q

Common symptoms of chronic leukemia slow onset in adult patients

A

-weakness/fatigue
-weight loss
-night sweats
-swollen abdomen (hepatosplenomegaly)
-infections
-easy bruising

82
Q

For Chronic Leukemia:
CML - high WBC - ______
CLL - high WBC - ________

A

neutrophils

lymphocytes

83
Q

what is the most common leukemia of adults in the Western world?

A

Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL)

84
Q

are Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) the same?

A

no, they have different origins – but they are grouped together because they are very similar otherwise

85
Q

describe the tumor that occurs in Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) and why

A

Indolent, slow-growing tumor (due to increased BCL-2), often asymptomatic

(remember, BCL-2 is anti-apoptotic - gradual accumulation of cells)

86
Q

how are Chromosomal translocations in Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) cured?

A

only achieved with hematopoietic stem cell transplant

BUT can only be treated once it reaches a certain threshold of division rate since therapies only target rapidly dividing cells and these tumors are slow

87
Q

some Chromosomal translocations in Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) tumors transform to what?

A

more aggressive diffuse large B-cell lymphoma (Richter transformation)

(prognosis = death within a year)

88
Q

What is a myeloproliferative disorder (neoplasm of mature cells of myeloid lineage) that particularly shows an increase in granulocytes

A

Chronic myeloid leukemia

89
Q

What chromosome and translocation is involved in chronic myeloid leukemia? What does it cause?

A

Philadelphia chromosome:

BCR-ABL t(9:22) translocation causing a fusion protein

90
Q

What is a blast crisis in chronic myeloid leukemia?

A

Some cases undergo transformation into an acute leukemia

91
Q

What is treatment for chronic myeloid leukemia?

A

Tyrosine kinase inhibitors (e.g. imatinib (Gleevec)) induce sustained remissions and prevent progression to blast crisis

92
Q

What does MDS stand for?

A

Myelodysplastic syndrome

93
Q

What happens in Myelodysplastic syndrome

A

Disordered maturation defect where bone marrow replaced by clonal, multipotent stem cells with capacity for differentiation into red cells (erythroid precursors), granulocytes and platelets

94
Q

T/F Myelodysplastic syndrome is usually idiopathic but can develop after chemotx or exposure to ionizing radiation

A

True

95
Q

In Myelodysplastic syndrome (MDS) the marrow is hypercellular/normocellular but the peripheral blood shows _______

A

cytopenias

96
Q

Myelodysplastic syndromehas a high risk of transformation to what?

A

transformation to AML (acute myeloid leukemia)

97
Q

What are some examples of B cell lymphomas?

A
  • Hodgkin Lymphoma
  • Non-Hodgkin Lymphomas
  • Plasma cell disorders: multiple myeloma
98
Q

What are some examples of Non Hodgkin lymphomas?

A
  • Follicular lymphoma
  • Marginal zone lymphoma
  • Diffuse Large B cell lymphoma
  • Burkitt lymphoma
99
Q

What type of cell often classifies Hodgkin lymphoma histologically?

A
  • Typified by the Reed-Sternberg (RS) cell which is a germinal center B cell
  • Some have EBV infection
100
Q

describe the histological appearance of Reed-Sternberg (RS) cell (B-cell)

A

Large cell with a multilobated nucleus, prominent nucleoli and which sometimes can be binucleated forming “owl eye” appearance

101
Q

What age is affected most by Hodgkin Lymphoma

A

Adolescents/young adults or patients >50 yrs

102
Q

what are the common signs/symptoms of Hodgkin Lymphoma?

A

-Painless lymphadenopathy (LAD)

-“B symptoms” - fever, weight loss, night sweats, pruritis, anemia

(underlined are associated with advanced disease)

103
Q

describe the classic Painless lymphadenopathy of Hodgkin Lymphoma?

A

arises in a single lymph node (lower cervical, supraclavicular, mediastinal LNs) → spreads to contiguous nodes

104
Q

what is Tx for for Hodgkin Lymphoma?

A

-Chemotherapy
-advanced disease also receives radiotherapy
-Immunotherapy (anti-PD-1 antibodies for refractory disease

105
Q

what is prognosis for Hodgkin Lymphoma?

A

high survival rate –> Five-year survival: >90%

106
Q

most Follicular lymphomas have what genetic characteristic?

A

t(14;18) translocation causing overexpression of BCL-2 protein

107
Q

what population are Follicular lymphomas seen in?

A

Adults >50 years

108
Q

what are the signs/symptoms of Follicular lymphomas? (1)

A

Painless, generalized lymphadenopathy

109
Q

how does the tumor of Follicular lymphomas appear?

A

Incurable, indolent lymphoma

110
Q

Treatment for Follicular lymphomas is reserved for ______ or symptomatic disease

A

bulky

111
Q

30-40% of Follicular lymphomas progress to what?

A

diffuse large B-cell lymphoma

112
Q

how will Follicular lymphomas appear histologically?

A

no discrete dark or light zones –> normal pattern/architecture destroyed due to tumor

113
Q

where do Extranodal marginal zone lymphomas arise from? what do they cause?

A

mucosa with lymphoid tissue (MALT) associated with epithelium (stomach, salivary glands etc.) → can cause swelling

114
Q

what sustains extranodal marginal zone lymphomas? (2)

A
  • chronic inflammation triggered by autoimmune disorders (Sjogren syndrome in salivary glands, or Hashimoto thyroiditis)
  • sites of chronic infection (H. pylori gastritis)
115
Q

how to treat H. pylori caused Extranodal marginal zone lymphomas and why?

A

-Kill the bug = kill the tumor
-bacteria allows specific T cells to drive growth and the survival of B cells
-BUT: polyclonal B cell growth can evolve into monoclonal change and spread to distant sites.

116
Q

how is localized Extranodal marginal zone lymphomas treated?

A

cured by simple excision followed by radiotherapy

117
Q

what is the most common type of lymphoma in adults?

A

Diffuse large B-cell lymphoma

118
Q

what are the genetic causes of Diffuse large B-cell lymphoma? (2)

A

-t(14;18) translocation of BCL-2

-translocations of MYC

119
Q

How does Diffuse large B-cell lymphoma often appear?

A

symptomatic, rapidly enlarging mass either within a lymph node or extranodal in virtually any organ or tissue

120
Q

Does the tumor of Diffuse large B-cell lymphoma grow slowly or quickly?

A

Aggressive tumor

121
Q

how to treat Diffuse large B-cell lymphoma?

A

intensive combination chemotherapy and anti-CD20 drugs (retuximab) with 60-80% complete remission

122
Q

how does Diffuse large B-cell lymphoma appear histologically?

A

diffuse wall to wall large cells with a lot of mitotic activity

123
Q

what is the Fastest growing human tumor?

A

Burkitt lymphoma

124
Q

what is the genetic cause of Burkitt lymphoma?

A

t(8;14) translocation of MYC and IgH (heavy chain)

125
Q

what populations is Burkitt lymphoma found in?

A

Effects mainly children and young adults usually at extranodal sites

126
Q

Where are jaw masses associated with EBV found?

A
  • African cases and about 20% of other cases associated with EBV
127
Q

what is a major symptoms of Burkitt lymphoma in north america?

A

-Abdominal masses

128
Q

describe treatment for Burkitt lymphoma

A

although the tumor is highly aggressive, with intensive chemotherapy most patients can be cured

129
Q

how does Burkitt lymphoma appear histologically?

A

“starry sky”

130
Q

pathologically, what occurs during Plasma cell neoplasms?

A

Neoplastic plasma cells secrete a monoclonal immunoglobulin (M protein) or immunoglobulin fragment which is a tumor marker and has pathologic consequences

131
Q

T/F M proteins are small and restricted to plasma

A

false

M proteins are large and restricted to plasma

132
Q

If immunoglobulin light chains are also made by Plasma cell neoplasms, they can be detected in what?

A

the urine and blood

133
Q

what is Dysproteinemia?

A

Abnormal protein content in the blood, usually related to immunoglobulins

134
Q

what is Monoclonal gammopathy (paraproteinemia, M component, M protein)?

A

an increase in a single immunoglobulin type as a result of a clone of plasma cells

135
Q

how are Abnormal immunoglobulins detected?

A

serum protein electrophoresis

136
Q

how does serum protein electrophoresis help to detect Abnormal immunoglobulins?

A

separates proteins based on charge, size and shape

137
Q

what are the 2 major types of protein in the serum?

A

albumin and globulin proteins (alpha-1, alpha-2, beta-1, beta-2 and gamma)

138
Q

what is increased serum albumin associated with? decreased?

A

-increased: severe dehydration

-decreased: malnutrition, cachexia, liver dz, nephrotic syndrome, protein-losing enteropathies, severe burns

139
Q

what is increased serum alpha-1 globulin associated with? decreased?

A

-increased: inflammatory states, pregnancy

-decreased: alpha-1 antitrypsin deficiency

140
Q

what is increased serum alpha-2 globulin associated with? decreased?

A

-increased: inflammatory states, nephrotic syndromes, oral contraceptive use, steroid use, hyperthyroidism

-decreased: hemolysis, liver disease

141
Q

what is increased serum gamma globulin associated with? decreased?

A

-increased: polyclonal and monoclonal gammopathies

-decreased: agammaglobulinemia, hypogammaglobulinemia

142
Q

what are Conditions with Abnormal Igs? (4)

A

-Multiple myeloma

-Monoclonal gammopathy of undetermined

significance (MGUS)

-Amyloidosis (light chain deposition with or without multiple myeloma)

-Waldenström’s macroglobulinemia (increased IgM blood levels in association with lymphoplasmacytic lymphoma)

143
Q

A common lymphoid malignancy with a Median age = 70 years (older adults)

A

Multiple myeloma

144
Q

what does Multiple myeloma involve in the body? what are symptoms?

A

bone marrow with associated lytic lesions (often ‘punched out’ radiolucenies) throughout the skeleton (vertebral column, ribs, skull etc.)

145
Q

what is the most frequent M protein seen in Multiple myeloma?

A

IgG

146
Q

what are Bence-Jones proteins?

A

kappa or lambda light chains that can be produced in Multiple myeloma – their small size allows excretion in the urine

147
Q

what is often the genetic cause of Multiple myeloma?

A

Often translocations fusing IgH locus (14, heavy chain) to cyclin D1 (11) and cyclin D3 genes

148
Q

What causes proliferation of cells in Multiple myeloma?

A

MM cells bind to bone marrow stromal cells causing them to make IL-6 which causes osteoclast precursor cells to proliferate

149
Q

In MM cells, up-regulation of ______ by bone marrow stromal cells activates osteoclasts → bone _____ → hypercalcemia, pathologic fracture

A
  • RANKL
  • resorption
150
Q

what is the mechanism that causes the following symptoms in Multiple myeloma?
-recurring bacterial infections

A

Defective production of normal B cells→ high risk for bacterial infections

151
Q

what is the mechanism that causes the following symptoms in Multiple myeloma?
-renal dysfunction

A

-obstructive proteinaceous casts (Bence-Jones proteins, complete immunoglobulin, albumin etc.)

-Light chain deposition in the glomerulus or

interstitial

-Hypercalcemia leads to dehydration and renal stones

-Bacterial pyelonephritis due to hypogammaglobulinemia

152
Q

how does pathologic fracture appear radiographically in Multiple myeloma?

A

“punched out” radiolucencies

153
Q

what can hypercalcemia lead to in Multiple myeloma?

A

-confusion, weakness, lethargy

154
Q

T/F renal failure can occur in up
to 50% of Multiple myeloma patient

A

true

155
Q

a definitive diagnosis of Multiple myeloma requires what?

A

bone marrow exam

156
Q

how does Multiple myeloma appear histologically?

A

note the plasma cells

157
Q

how is Multiple myeloma treated?

A

-proteasome inhibitors (Chemotx)
-Bisphosphonates

158
Q

what are Bisphosphonates and what do they play in treating Multiple myeloma?

A

drugs that inhibit bone resorption –> reduce fractures and hypercalcemia

159
Q

without treatment, what is the prognosis for Multiple myeloma?

A

death within a year

160
Q

with treatment, what is the prognosis for Multiple myeloma?

A

Median survival: 4-7 years

161
Q

Some Multiple myeloma patients have this form that may be asymptomatic for many years

A

“smoldering myeloma”

162
Q

what is another name for Mycosis fungoides?

A

Cutaneous T-cell lymphoma

163
Q

what are symptoms of Mycosis fungoides?

A

-Erythematous rash progressing to a plaque and then to a tumor phase.
-Can spread to lymph nodes and viscera

164
Q

when Mycosis fungoides becomes systemic, how does it present?

A

-tumor cells in peripheral blood = Sezary syndrome - 1-3 yr survival

165
Q

what causes Adult T-cell leukemia/lymphoma?

A

human T-cell leukemia virus type I (HTLV-1)

166
Q

where is Adult T-cell leukemia/ lymphoma edemic?

A

southern Japan, Caribbean, West Africa

167
Q

what is prognosis for Adult T-cell leukemia/lymphoma?

A

very aggressive with median survival time – approx. 8 months

168
Q

what is Histiocytosis?

A

a variety of proliferative disorders of dendritic cells or macrophages

169
Q

what do Histiocytic neoplasms range from? what is between these two extemes

A

-highly malignant rare neoplasms to benign/reactive proliferations in lymph nodes
-Between these extremes is Langerhans cell histiocytosis (histiocytosis X)

170
Q

what are Langerhans cells? what do they do?

A

-Immature dendritic cells found in the epidermis with similar cells found in many other organs.
-capture antigens and present them through MHC class II to T cells

171
Q

Proliferating cells in Langerhans cell histiocytosis (LCH) appear how?

A
  • more like tissue macrophages (histiocytes) rather than dendritic cells.
  • Eosinophils also present
172
Q

What is the pathogenesis of Langerhans cell histiocytosis?

A

BRAF mutation (component of RAS signaling pathway

173
Q

Langerhans cell histiocytosis is acute/chronic presentations that affect what?

A

skin, viscera and/or bone

174
Q

What is the most common clinical manifestation of langerhans cell histiocytosis?

A

bone involvement (eosinophilic granuloma of bone)

175
Q

Which bones are most affected from langerhans cell histiocytosis?

A

Skull, ribs, vertebrae and mandible are commonly affected (similar locations as multiple myeloma)

176
Q

What are symptoms of bone involvement in langerhans cell histiocytosis?

A
  • Dull pain and tenderness often present
  • Punched-out or ill-defined radiolucency
177
Q
A

General Pathology (32 decks)

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