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Heme and Lymph > WBC 3 > Flashcards

WBC 3 Flashcards

1
Q

Plasma cell neoplasms are B cell proliferations that virtually always secrete what?

A

monoclonal Ig or Ig fragment

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2
Q

what are the plasma cell neoplasms/dyscrasias and related disorders discussed?
ALL CAPS the common ones

A
  • MULTIPLE MYELOMA
  • Waldenstrom Macroglobulinemia
  • heave chain disease
  • Primary or Immunocyte-associated amyloidosis
  • MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE (MGUS)
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3
Q

Explain clusters and infiltrates of plasma cells?

A

If you see clusters of plasma cells then you sorry about discrasias but if you see infiltrates of them on biopsy it doesn’t always mean dyscrasia because they are a feature of chronic inflammation

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4
Q

what is the most common and deadly of the plasma cell dyscrasias

A

Mutliple myeloma

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5
Q

describe myeloma kidney

A

Bence jones proteins are light chains that deposit in kidney and are toxic to proximal tubular epithelial cells

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6
Q

describe the lytic bone lesions of Multiple myeloma

A
  • usually skull, vertebral bodies, ribs
  • can be almsot anywhere
  • tend to be in upper portion of body
  • Not generally in long bones
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7
Q

Describe the rouleaux formations of multiple myeloma

A
  • RBCs seem to stack on one another
  • caused by increased viscosity of blood
  • due to elaboration of acute phase reactants like fibrinogen or fibrin
  • decreases surface tension
  • NOT pathopneumonic . . also seen in autoimmune disease like RA
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8
Q

Differential Dx for hypercalcemia

A
  • MM
  • Sarcoid
  • Vitamin D intoxication
  • milk alkali syndrome
  • hyperparathyroidism
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9
Q

What are the bizarre multinucleated plasma cells in MM

A
  • Flame cells

- Mott cells with multiple grapelike cytoplasmic droplets with inclusions

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10
Q

What are the inclusions in a Mott cell of MM

A
  • Russell bodies if cytoplasmic

- Dutcher bodies if nuclear

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11
Q

2 nuclei in plasma cell . . cytoplasmic inclusions which are cluster if immunoglobulin

A

think multiple myeloma

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12
Q

What is the most common manifestation of MM

A

IgG with kappa light chains . . monoclonal

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13
Q

diagnosis of MM

A

-More than 3 gm/dL of serum Ig and/or more than 6 mg/dL of (urinary) Bence-Jones protein

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14
Q

What is the most common monoclonal Ig (“M protein) in MM?

A
  • IgG (55%)

- then IgA (25%)

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15
Q

Excessive production and aggregation of M protein in MM of what types leads to symptoms related to hyperviscosity

A

IgA and/or IgG3 subtype

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16
Q

What % of myelomas are nonsecretory?

What do they still have?

A
  • 1%

- lytic bone lesions

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17
Q

an infrequent variant of MM that presents as a single mass in bone or soft tissue
-what are the 2 types

A

solitary myeloma (plasmacytoma)

  • osseous/intraosseous
  • another that is soft tissue and not bone
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18
Q

an uncommon variant of MM defined by a lack of symptoms and a High plasma M component (> 3 gm/dL)
-No bony lesions and no hypercalcemia

A

-Smoldering myeloma

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19
Q

Epidemiology of MM

A
  • higher in men and Africans

- older adults . . peak of 65-70

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20
Q

important clinical features of MM

A
  • Bone resorption –> pathological fractures and chronic pain
  • Hypercalcemia –> confusion, weakness, lethargy, constipation, and polyuria and contributes to renal dysfunction
  • Decreased normal Igs –> recurrent bacterial infections
  • Cellular immunity is NORMAL
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21
Q

explain Solitary myelomas (Plasmacytomas) and the progression to MM

A
  • Osseous inevitably progress to MM but can take 10-20 years
  • Extraosseous (extramedullary), particularly of upper respiratory tract are frequently cured by local resection and don’t tend to progress to MM
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22
Q

entity that is a middle ground between multiple myeloma and monoclonal gammopathy of uncertain significance

A

Smoldering myeloma

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23
Q

What is the most common plasma cell dyscrasia. NOT neoplasm`

A

MGUS

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24
Q

Serum M protein level for MGUS

A

LESS than 3 gm/dL

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25
Q

What % of MGUS pts develop a symptomatic plasma cell neoplasm?

A

1%

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26
Q

How do you predict if smoldering myeloma or MGUS will progress to MM

A

you can’t . .it’s unpredictable. you need periodic assessment of serum M component levels and Bence Jones proteinuria

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27
Q

what translocation is good outcome for MM?

more aggressive?

A
  • Cyclin D1

- Deletions of 13q, 17p, and t(4;14)

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28
Q

age for lymphoplasmacytic lymphoma

A

a B cell neoplasm of older adults .. usually 6th or 7th decade

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29
Q

Clinical features of Lymphoplasmacytic Lymphoma

A
  • plasma cell component secretes monoclonal IgM, often in ammounts sufficient to cause a hyperviscosity syndrome known as Waldenstrom macroglobulinemia
  • Complications from free light chains relatively rare
  • Bony lytic lesions DO NOT occur
  • COLD AGGLUTININS
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30
Q

hyperviscosity syndrome of lymphoplasmacytic lymphoma

A
  • visual impairments
  • Neurological problems
  • Bleeding
  • Cryoglobulinemia . . Taynaud phenomenon and bold uticaria
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31
Q

Treatment of lymphoplasmacytic lymphoma

A

incurable

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32
Q

increased number of what cells in lymphoplasmacytic lymphoma . . besides the B cells

A

Mast cells . . tissue macrophage

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33
Q

Epidemiology of Mantle Cell Lymphoma

A
  • 5th or 6th decades

- male predominance

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34
Q

The mutation in mantle cell lymphoma leads to overexpression of what?

A

cyclin D1 . . promotes G1 to S phase progression

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35
Q

Most common presentation for Mantle cell lymphoma

A

Painless lymphadenopathy

-SPleen and gut symptoms also common

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36
Q

Prognosis and survival in Mantle cell lymphoma

A

Poor . . median survival 3 to 4 years

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37
Q

What variant of mantle cell lymphoma is associated with even shorter survival

A

Blastoid variant and a “proliferative” expression

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38
Q

Transformation of mantle cell lymphoma

A

can overtime to DLBCL

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39
Q

Morphology of mantle cell lymphoma

A

Expanding population of neoplastic cells resemble mantle cells , but NO PROLIFERATION CENTERS as in CLL/SLL

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40
Q

Mantle cell lymphoma has lymphomatoid polyposis. . . Tell me what this is

A

mucosal involvement of the small bowel or colon produces polyp-like lesions and makes you think it is IBD

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41
Q

Encompasses a heterogeneous group of B cell tumors that arise within lymph nodes, spleen or extranodal tissues . .the latter were originally notes at mucosal sites so thus often called “maltomas” . . especially in GI tract

A

Marginal Zone lymphoma

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42
Q

These arise in extranodal tissues exhibiting chronic inflammation of autoimmune or infectious etiology. may regress if inciting agents are eliminated
Give examples

A

Marginal Zone Lymphoma

  • Salivary - Sjogren Syndrome
  • Salivary - Hashimoto’s thyroiditis
  • Stomach - Helicobacter
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43
Q

Explain pathogenesis of Marginal Zone lymphoma

A
  • Arise in chronically inflamed tissues that are in a continuum b/t reactive hyperplasia and full-blown lymphoma.
  • They begin as polyclonal and acquire initiating mutations
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44
Q

Dry tap

A

Hairy cell leukemia

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45
Q

Rare but distinctive B cell neoplasm is predominantly a disease of middle aged white males. Median age 55. Male to female ratio 5:1. characteristic image seen on phase contrast microscopy

A

Hairy cell leukemia

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46
Q

What causes the dry tap in hairy cell leukemia

A

cells produce an ECM of reticular fibers and this meshwork acts like chronic inflammatory fibrosis so when you try to aspirate the bone marrow you can’t get anything

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47
Q

Clinical manifestations of Hairy Cell leukemia

A
  • Splenomegaly (often massive) is most common and sometimes only abnormal physical finding
  • Hepatomegaly is less frequent and lymphadenopathy is rare
  • Pancytopenia and splenic sequestration is seen in over half
  • about 1/3 present with infections
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48
Q

Hairy cell leukemia has increased incidence of what and due to what?

A

atypical mycobacteria due to unexplained monocytopenia

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49
Q

What is on the surface of hairy cell leukemia cells that is helpful

A

IgG

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50
Q

What are the peripheral T cell and NK cell neoplasms

A
  • Peripheral T cell lymphoma, unspecified (NOS)
  • Anaplastic Large cell lymphoma (ALK pos)
  • Adult T cell leukemia/lymphoma
  • Mycosis Fungoides/Sezary Syndrome
  • Large Granular Lymphocytic leukemia
  • Extranodal NK/T cell lymphoma
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51
Q

Clinical symptoms of Peripheral T cell lymphoma, unspecified

A
  • Lymphadenopathy
  • sometimes eosinophilia
  • pruritis,
  • Fever
  • Weight loss
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52
Q

What is the prognosis of peripheral T cell lymphoma unspecified?

A

significantly worse than B cell analogues

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53
Q

An uncommon entity defined by the presence of rearrangements in the ALK gene?
What chromosome is this?

A

Anaplastic Large Cell lymphoma

  • chromosome 2p23
  • ALK is not expressed in normal lymphocytes or other lymphomas so its detection is a reliable indicator
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54
Q

These tumor cells tend to cluster about venules and infiltrate lymphoid sinuses, mimicking the appearance of metastatic carcinoma

A

Anaplastic Large Cell lymphoma

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55
Q

Age for Anaplastic Large cell lymphoma

A

tend to occur in children or young adults

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56
Q

How do Anaplastic large cell lymphomas present?
Prognosis?
Age dependent prognosis?

A

soft tissue “masses”

  • very good prognosis unlike other aggressive peripheral T cell neoplasms
  • Morphologically similar lymphomas but WITHOUT ALK rearrangements occur in adults and have a poor prognosis
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57
Q

“Hallmark” horseshoe like or embryoid nuclei and abundant cytoplasm

A

Anaplastic large cell lymphoma

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58
Q

How do you diagnose Peripheral T cell lymphoma, unspecified

A

basically by saying its none of the other peripheral T cell neoplasms but has T cell markers

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59
Q

A neoplasm of CD4+ T cells only observed in adults infected with HTLV-1. occurs mainly where the virus is endemic i.e. Japan, W. Africa, and the Caribbean basin

A

Adult T-cell leukemia/lymphoma

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60
Q

What may adult T cell leukemia/lymphoma cause?

A

a progressive demyelinating disease of the CNS and spinal cord associated with tropical spastic paraparesis

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61
Q

tumor of CD4+ helper cells that home to the skin . . plaquelike manifestations

A

Mycosis Fungoides/Sezary Syndrome

62
Q

Describe the Sezary Syndrome that comes with Mycosis Fungoides?

A

-A variant of generalized exfoliative erythroderma with an associated leukemia of “Sezary” cells with characteristic CEREBRIFORM NUCLEI

63
Q

tumor cells are large lymphocytes with abundant blue cytoplasm and scant coarse azurophilic granules

A

Large Granular lymphocytic leukemia

64
Q

What are the 2 variants of Large Granular lymphocytic leukemia

A

T cell and NK

65
Q

What cell indices dominate the clinical picture of Large Granular lymphocytic leukemia

A

Neutropenia and anemia

66
Q

What syndrome is associated with Large Granular lymphocytic leukemia and describe the syndrome

A

Felty syndrome: RA, splenomegaly and neutropenia

67
Q

This peripheral T and NK cell neoplasm is highly associated with EBV and typically presents as a highly destructive nasopharyngeal mass . . less commonly involved the testes and/or skin

A

Extranodal NK/T cell lymphoma

68
Q

Tends to surround and invade small vessels leading to extensive ischemic necrosis.
-Rare in US but 3% of NHL is Asia

A

Extranodal NK/T cell lymphoma

69
Q

What is odd about the association of EBV and extranodal NK/T cell lymphoma concerning it’s cell markers

A

it does NOT express CD21 which is the receptor for EBV

70
Q

Average age at diagnosis of Hodgkin lymphoma

A

32

71
Q

Describe the difference b/t HL and NHL

A
  • NHLs frequently occur at extranodal sites and spread in an unpredictable fashion; multiple peripheral nodes; Noncontiguous spread; Waldeyer ring and mesenteric Nodes COMMONLY involved
  • HL arises at a single node or chain of nodes (Cervical, mediastinal, para-aortic) and spreads first to contiguous lymphoid tissues; Mesenteric nodes and Waldeyer ring RARELY involved; extranodal presentation RARE
72
Q

Reed Sternberg cell

A

Hodgkin lymphoma

73
Q

explain what Reed Sternberg cells do

A

release a wide variety of cytokines, chemokines, and other factors that induce the accumulation of REACTIVE lymphocytes, macrophages, and granulocytes. they are aneuploidy and possess a diverse clonal chromosomal aberrations

74
Q

A large cell with 2 nuclei and extremely large nucleoli with clearing around it and relatively abundant cytoplasm . . in a background of mononuclear cells that are NOT neoplastic but reactive . . the cell itself is neoplastic

A

RS cell

75
Q

What are the 4 “classic” types of Hodgkin lymphoma . . what makes them classic?

A
  • Nodular sclerosis
  • Mixed cellularity
  • lymphocyte rich
  • lymphocyte depletion

-the RS cells have similar immunophenotypes (CD30+)

76
Q

Describe the Nodular sclerosis type of Hodgkin

A
  • most common
  • uncommonly assoc. with EBV
  • equal gender involvement
  • Propensity for lower cervical, supraclavicular sites
  • especially noted for frequent mediastinal involvement
  • prognosis excellent
77
Q

Describe the mixed cellularity type of Hodgkin

A
  • RS cells and variants usually abundant
  • RS cells infected by EBV in 7-%
  • more common in males
  • more commonly Biphasic in age
  • Systemic symptoms (night sweats and weight loss)
  • advanced tumor stage but prognosis is very good
78
Q

Describe the lymphocyte-rich type of Hodgkin

A
  • uncommon
  • EBV in 40%
  • very good to excellent prognosis
79
Q

Describe the lymphocyte depletion type of Hodgkin

A
  • Least common
  • immunophenotyping essential as it must be distinguished from NHL .. . doesn’t have background of reactive cells
  • > 90% EBV involvement
  • predominantly elderly, in HIV+ of any age, and in non-industrialized countries
  • overall outcome less favorable than other classic types
80
Q

what is the NON classic type of Hodgkin

A

Lymphocyte predominance

81
Q

Describe the lymphocyte predominance type of Hodgkin

A
  • uncommon
  • different immunophenotype
  • L & H cells (Popcorn cells)
  • 3-5% transform into a tumor resembling a diffuse, Large B cell lymphoma
  • EBV Is NOT associated with this type
  • CD20+, CD15 and 30 neg,
  • young males with cervical or axillary lymphadenopathy; mediastinal
82
Q

What are the most common subtypes of NHL in people with HIV/AIDS

A

primary central nervous system lymphoma

83
Q

What are the “AIDS defining illnesses”

A
  • Kaposi’s sarcoma
  • NHL
  • cervical cancer
84
Q

what expands in a virally infected lymph node with EBV

A

paracortex

85
Q

In an HIV patient with normal T4 cell count that has a virally infected lymph node, what is seen? describe what this is

A

Brisk follicular hyperplasia

-its a B cell response

86
Q 
What cytokines do TH1 cells produce?
What induces this subset?
What do the cytokines released do? 
What do these defend against?
What is it's role in disease?
A
  • IFN-gamma
  • IFN-gamma, and IL-12
  • Macrophage activation, Stimulation of IgG, antibody production
  • Intracellular microbes
  • Immune mediated chronic inflammatory diseases (often autoimmune)
87
Q 
What cytokines do TH2 cells produce?
What induces this subset?
What do the cytokines released do? 
What do these defend against?
What is it's role in disease?
A
  • IL-4, 5, 13
  • IL-4
  • stimulation of IgE, activations of mast cells and eosinophils
  • Helminthic parasites
  • Allergies
88
Q 
What cytokines do TH17 cells produce?
What induces this subset?
What do the cytokines released do? 
What do these defend against?
What is it's role in disease?
A
  • IL-17 and 22
  • TGF-beta, Il-6, IL-1, IL, 23
  • recruitment of neutrophils and monocytes
  • extracellular bacteria and fungi
  • Immune mediated chronic inflammatory diseases (often autoimmune)
89
Q

for these you do not need blasts in the peripheral blood but instead in bone marrow

A

AML; acute myeloid leukemia

90
Q

What are the 3 broad categories of myeloid neoplasm and generally describe them

A
  • AML; an accumulation of immature myeloid forms (blasts) in the bone marrow suppresses normal hematopoiesis
  • Myelodysplastic syndromes (MDS): ineffective hematopoiesis leads to cytopenias
  • Myeloproliferative disorders (MPD) there is usually increased production of one or more types of committed/mature white/red cells of the myeloid series
91
Q

The diagnosis of AML is based on the presence of what

A

at least 20% myeloid blasts in the bone marrow

-Bone marrow exam is required if AML is suspected

92
Q

What has worse prognosis MDS or MPD

A

generally MDS

93
Q

MDS and MPD —-> AML

A

they often “transform” to AML

94
Q

What are the WHO classifications of AML?

A

I: AML with genetic abberrations
II: AML with MDS-like features
III: AML, therapy related
IV: AML, NOS

95
Q

What are the subtypes of MDS

A
  • RA: refractory anemia
  • RARS: above with ring sideroblasts
  • RAEB: . . . excess blasts
  • RAEB-t . . same as above but therapy related
  • CMML: Chronic myelomonocytic leukemia (not actually a leukemia but can transform to AML)
  • MDS, NOS
96
Q

What are the subtypes of MPD?

A
  • CML: Chronic myelocytic leukemia (not a leukemia but can transform to AML
  • PV: polycythemia vera
  • ET: essential thrombocytosis
  • PM: progressive myelofibrosis
97
Q

Give me the type I WHO AML genetic aberrations with their prognosis and FAB class

A
  • t(8;21) . . M2 . . Favorable
  • Inv(16) . . M4eo . . Favorable
  • t(15;17) . . M3 (APL . . DIC) . . Intermediate
  • t(11q23;v) . . M4,M5 . .. Poor
98
Q

Give me the type II WHO AMLs with prognosis

A
  • with prior MDS syndrome . . very poor

- W/O prior myelodysplastic syndrome . . poor

99
Q

What is the prognosis of AML, therapy related

A

Very poor

100
Q

M0

A
  • AML, minimally differentiated
  • Blast lack definitive cytology and cytochemic markers of myeloblasts (MPO neg)
  • express myeloid lineage antigens and resemble myeloblasts ultrastructurally
101
Q

M1

A
  • AML, without maturation
  • > 3% of blasts are MPO+
  • few granules or Auer rods and little maturation beyond myeloblast stage
102
Q

M2

A
  • AML with myelocytic maturation (Most common)
  • Full range of myeloid maturation through granulocytes
  • Auer rods present in most
  • often t(8;21)
103
Q

M3

A
  • APL
  • most cells are hypergranular promyelocytes
  • often with many Auer rods per cell
  • younger pts (35-40)
  • DIC
  • t(15;17)
104
Q

M4

A
  • AML with myelomonocytic maturation
  • myelocytic and monocytic diff.
  • MPO+
  • monoblasts are + for nonspecific esterases
  • inv(16)
105
Q

M5a/b

A
  • AML with monocytic maturation

- MPO- but nonspecific esterase+

106
Q

M6a/b

A
  • AML with erythroid maturation

- dysplastic erythroid precursors

107
Q

M7

A

-AML with megakaryocytic maturation

108
Q

Cell markers of maturity for AML

A
  • CD34: precursor/immature
  • CD64: mature myeloid
  • CD33: immature
  • CD15: more mature
109
Q

Clinical features of AML

A
  • similar to ALL
  • Fatigue (anemia), fever/infection (neutropenia), bleeding (thrombocytopenia)
  • petechiae/echymoses/mucosal hemorrhages/hematuria
110
Q

infections and AML

A

opportunistics: fungi, pseudomonas, commensals, pneumocystis

111
Q

Tissue and AML

A

-skin/gingiva may be prominent especially with monocytic differentiation

112
Q

localized tissue mass and AML

A

Granulocytic sarcoma

113
Q

CNS and AML

A

spread occues but less commonly than ALL

114
Q

Leukemia Cutis

A

AML . . monocytic type

115
Q

prognosis of therapy related MDS

A

Worse

116
Q

Blast % in MDS

A

<20%

117
Q

Clinical features of MDS

A
  • older adults (mean 70)
  • progression to AML in 10-40%
  • cytopenias more severe in pts with t-MDS with more rapid progression to AML and survival f only 4-8 months
118
Q

Ring sideroblasts

A

MDS

119
Q

Pseudo-Pelger-Huet cells

A

-neutrophils with only 2 nuclear lobes . . MDS

120
Q

Pawn Ball megakaryocytes

A

Megakaryocytes with single nuclear lobes or multiple separate nuclei . . MDS

121
Q

What is the pathogenic feature of MPD

A

presence of mutated, constitutively activated tyrosine kinases

122
Q

Extramedullary hematopoiesis and MPD

A

common

123
Q

Explain the spent phase of MPD

A

progression but not to AML. similar to hairy cell leukemia that generates extracellular reticulin and fibrils. All the cytokines and chemokines get elaborated and cause fibrinogenic effects . . . looks like MDS

124
Q

CML is distinguished from of MPDs by the presence o what?

A

BCR-ABL . . chrom. 22 and 9 . . Philadelphia chromosome

-GOOD PROGNOSIS

125
Q

Clinical features of CML

A
  • Adults . . 50 . .m>F
  • insidious onset
  • fatigue, weakness, weight loss, anorexia
  • Abdominal fullness/pain (splenomegaly due to extramedullary hematopoiesis.
  • If LUQ pain . .may be splenic infarct
126
Q

Diagnosis of CML

A
  • Leukocytosis; may be > 100,000
  • <10% blasts
  • bone marrow markedly hypercellular
127
Q

Sea blue histiocytes and increased reticulin

A

CML

128
Q

LAP utilization of CML

A
  • Low in CML

- High in reactive leukamoid reactions

129
Q

Prognosis of CML

A
  • 3 years survival without treatment
  • 50% enter an “accelerated” phase which after 6 to 12 months enter a “blast crisis”
  • other 50% develop and abrupt “blast crisis” with no accelerated phase
130
Q

characterized by increased marrow production of red cells, granulocytes, and platletes (panmyelosis). but, the increase in red cells (polycythemia) is responsible for most of the symptoms in the disorder

A

Polycythemia vera

131
Q

erythropoietin levels in PV

A

significantly suppressed

132
Q

Clinical features of PV

A
  • adults
  • insidious onset; plethora, cyanosis, intense pruritus
  • HCT>55%
  • symptoms of hyperviscosity, major bleeding and thrombotic episodes
  • Leukocytosis and thrombocytosis (often >500,000)
  • about 25% first come to medical attention due to DVT, MI, or stroke
  • Hyperuricemia common . . gout
133
Q

Treatment of PV

A
  • phlebotomy

- JAK2 inhibitors

134
Q

Thrombocytosis WITHOUT polycythemia

A

Essential thrombocytosis (ET)

135
Q

ET is often associated with activating point mutations in what?

A

JAK2 (50%) or MPL (5-10%), a receptor tyrosine kinase that is normally activated by thrombopoietin

136
Q

Clinical features of ET

A
  • adults>60 usually

- Often platelet forms are enlarged/abnormal

137
Q

What is necessary for diagnosis of ET

A
  • Bone Marrow biopsy
  • megakaryocytes are substantially increased
  • Thrombotic and bleeding
138
Q

survival in ET

A

12 to 15 years

139
Q

treatment of ET

A

chemo

140
Q

progression to AML in ET

A

uncommon

141
Q

The hallmark of this MPD is the development of obliterative marrow fibrosis by non-neoplastic fibroblasts

A

Primary myelofibrosis

142
Q

Clinical features of primary myelofibrosis

A
  • Least common MPD and difficult to treat
  • > 60
  • early prominent megakaryopoiesis and granulopoiesis. often extensive EMG
  • Abnormal megakaryocytes produce PDGF and TGF-beta, powerful fibroblast mitogens
  • Activating JAK2 in 50-60%; MPL in 1-5%
  • Prominent Splenomegaly
143
Q

Spent phase and primary myelofibrosis

A
  • most commonly goes to spend phase
  • obliterates marrow leading to cytopenias and EMH
  • aka “agnogenic myeloid metaplasia
144
Q

blood smear in primary myelofibrosis

A

Normochromic normocytic anemia

-leukoerythroblastic

145
Q

Prognosis for primary myelofibrosis

A

-3-5 years

146
Q

Vesicular nuclei with linear grooves or folds and vacuolated cytoplasm . ..Birbeck granules in cytoplasm containing Langerin

A

Langerhans Cell Histiocytosis

147
Q

What are the 3 diseases in the category of Langerhans Cell Histiocytosis

A
  • Multifocal/multisystemic Langerhans Histiocytosis (Letterer-Siwe disease)
  • Unifocal and multifocal unisystem Langerhans cell histocytosis (eosinophilic granuloma)
  • Pulmonary Langerhans cell histrocytosis
148
Q

Describe Letterer-Siwe disease

A
  • before 2 years
  • cutaneous lesions resembling a seborrheic eruption
  • Fevers, infections
  • Bone lesions
  • With chemo, 50% 5 year survival
  • rapidly fatal if untreated
149
Q

Describe the unifocal and multifocal eosinophilic granulomas

A
  • Unifocal: skeletal system of older children. most commonly calvarium, ribs, femur
  • multi: multiple erosive bony masses in young children; 50% have diabetes insipidus; Hand-Schuller-Christian triad: Calvarial bone defects, diabetes insipidus, and exophthalmos
150
Q

describe treatment of eosinophilic granulomas

A
  • many spontaneously regress
  • chemo if multifocal
  • Local excision or irradiation of unifocal
151
Q

What does Pulmonary Langerhans cell histiocytosis present as?
What lobes?
Neoplastic if what mutations?
associated with what environmental factor?
Age?
Progression?

A
  • bilateral interstitial disease
  • Middle and upper
  • BRAF
  • cigarette smoking
  • typically adults
  • May regress spontaneously on cessation of smoking

Heme and Lymph (27 decks)

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