Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Heme/Lymph - Exam 1 > Myeloid Stuff - SRS > Flashcards

Myeloid Stuff - SRS Flashcards

1
Q

What are the intermediate steps between a myeloid stem cell and a red cell?

(Just the ones shown on the third slide image)

A
  1. Myeloid stem cell
  2. pronormoblast
  3. basophilic normoblast
  4. polychromic normoblast
  5. orthochromatic normoblast
  6. Red cell
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

In that same image, what are the steps form myeloid stem cell to megakaryocyte?

A
  1. myeloid stem cell
  2. megakaryoblast
  3. promegakaryocyte
  4. megakaryocyte
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the steps from myeloid stem cell to monocyte?

A
  1. Myeloid stem cell
  2. monoblast
  3. promyelocyte
  4. monocyte
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the steps from a myeloid stem cell to a segmented neutrophil?

A
  1. Myeloid stem cell
  2. myeloblast I
  3. Myeloblast II
  4. Promyelocyte
  5. Neutrophil myelocyte
  6. neutrophil metamyelocyte
  7. Neutrophil Band
  8. Segmented Neutrophil
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the two paths to an eosinophil?

A
  1. Myeloid stem cell
  2. Myeloblast I OR myeloblast II
  3. Eosinophil precursor
  4. Eosinophil
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the two paths to a mature basophil?

A
  1. Myeloid stem cell
  2. myeloblast type I
  3. Myeloblast type II
  4. Basophil precursor
  5. Mature basophil
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Myeloid neoplasms arise from hematopoietic stem cells that give rise to cells of what lineages?

A

Myeloid - erythroid, granulocytic, thrombocytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the three categories of myeloid neoplasia?

A
  1. Acute myelogenous leukemias
  2. Myelodysplastic syndrome
  3. Chronic myeloproliferative disorders
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How is an acute myelogenous leukemia defined?

What would you call a soft tissue mass of these cells?

A

•>20% immature progenitor cells accumulate in the bone marrow

Myeloid (granulocytic) sarcoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are myelodysplastic syndromes associated with?

A

Ineffective hematopoiesis and resultant peripheral blood cytopenias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Describe the following for both acute and chronic myelogenous leukemias.

  1. Cell maturity
  2. Clinical prognosis
  3. Population affected most often
A

Acute

  1. Immature/blasts
  2. live months
  3. kids and adults

Chronic

  1. Maturation present
  2. live years
  3. adults
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What advantages do class one mutations typically confer on tumor cells?

A

Proliferation and survival

(not impacting differentiation)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What do class II mutations typically confer to cells?

A

Impaired haematopoietic differentiation and subsequent apoptosis (pretty sure this statement is saying that apoptosis is prevented)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the two genetic aberrations associated with favorable outcomes in AML?

What fusion genes are associated with this?

A
  1. t(8;21) (q22,q22) - RUNX1/ETO fusion gene
  2. INV(16) (P13;q22) - CBFβ/MYH11
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the mutation and its corresponding fusion gene we should be aware of for APL (AML in his table, but changed by WHO to APL)?

A

t(15;17)(q22;11-12); RARα/PML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

The t(15;17) translocation APL produces the RARα/PML fusion gene. The prognosis in this condition is intermediate since we can use Tretinoin to force differentiation.

What are the key morphology and presentation components we should know about this condition?

A
  1. progranulocytes with numerous bundled auer rods
  2. High incidence of DIC
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the prognosis for AML with MDS-like features in the following settings?

  1. Prior MDS
  2. AML with multilineage dysplasia
  3. AML with MDS0like cytogenetic aberrations
A

All poor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

If a patient develops AML secondary to radiation therapy or alkylator therapy, what is their prognosis.

A

Exceedingly poor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is “specific esterase” associated with?

A

Neutrophils

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is “nonspecific esterase” associated with?

A

Monocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Classify the follow description according to the FAB system:

Full range of myelocytic maturation

A

M2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Classify the follow description according to the FAB system:

>3% of blasts positive for myeloperoxidase

A

M1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What FAB class is described here?

Full range of myelocytic maturation

A

M2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is the M3 class of AML?

A

APL: PML-RARa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

FAB class?

Negative for myeloperoxidase; myeloid antigens detected on blasts by flow cytometry

A

M0

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What FAB clasS?

Erythroid/myeloid subtype defined by >50% dysplastic maturing erythroid precursors and >20% myeloblasts

A

M6A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

FAB class?

pure erythroid subtype defined by >80% erythroid precursors without myeloblasts

A

(M6b)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

FAB class?

nonspecific esterase-positive monoblasts and pro-monocytes predominate in marrow and blood;

A

M5A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

FAB class?

mature monocytes predominate in the blood

A

M5b

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

FAB classes?

  • Erythroid/myeloid subtype defined by >50% dysplastic maturing erythroid precursors and >20% myeloblasts;
  • pure erythroid subtype defined by >80% erythroid precursors without myeloblasts
A

M6a

M6b

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

FAB class?

Blasts of megakaryocytic lineage predominate; detected with antibodies against megakaryocyte-specific markers (GPIIb/IIIa or vWF); often associated with marrow fibrosis;

A

M7

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What is the most common AML in Down Syndrome?

A

M7

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Assign FAB classes:

  1. AML with myelocytic maturation
  2. AML with myelomonocytic maturation
  3. AML with erythroid maturation
  4. AML with monocytic maturation
  5. AML with megakaryocytic maturation
  6. AML without maturation
  7. AML, minimally differentiated
  8. APL
A
  1. M2
  2. M4
  3. M6a/b
  4. M5a/b
  5. M7
  6. M1
  7. M0
  8. M3
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What is the median age at dx of acute myelogenous leukemia?

A

67

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What is the median age3 at dx for acute lymphoblastic leukemia?

A

14 (did I get you with the three? typo that I left in to try and mess with you… har har

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What is the finding indicated in the image?

What is it pathognomonic for at our level?

A

Auer Rods - azurophilic granules in linear arrangement

Pathognomonic for AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Chromosomal abnormalities occur in 90% of AML patients when using special high res. banding techniques. 50-70% show karyotypic changes.

Chromosomal abnormalities, both types and overall frequency, are increasingly used to guide therapy and predict prognosis

  1. What is the translocation in APL?
  2. tx?
  3. What are two translocations associated with a poor prognosis?
A

t(15;17)

All trans-retinoicacid (tretinoin)

Arsenic salts - degrade PML/RARA fusion protein

5q- and 11q

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

AML presents with same acute onset and symptoms as ALL (anemia, infection, fever, bone pain, and bleeding). Neoplastic cells with monocytic differentiation infiltrate the skin.

What is the prognosis vs ALL?

What is one important characteristic to remember about the possible complications of AML?

A

Poorer than ALL by a lot

DIC occurs in the AML with t(15;17) RARα/PML fusion gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What percent of AML patients achieve complete remission?

A

60% achieve one complete remission

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What percent of AML patients who experience remission remain disease free after 5 years?

A

15 - 30%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What type of leukemia is myeloid leukemia most often?

A

Acute megakaryoblastic leukemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What are the physical findings in AML?

Give me 5

A
  1. •Splenomegaly
  2. •Hepatomegaly
  3. •Gum swelling or skin nodules
  4. •Sternal tenderness
  5. •Petechiae
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What is the median age at dx of AML?

A

67

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What is the median age of death for AML?

A

72

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

In which types of AML is the gum swelling/skin nodules most common?

A

Monocytic types

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

There are a number of myelodysplastic syndromes (MDS)

List as many as you can, but be sure to nail the four he highlighted.

A

•Refractory cytopenia with unilineage dysplasia

• Refractory anemia

•Refractory neutropenia

•Refractory thrombocytopenia

  • Refractory anemia with ring sideroblasts
  • Refractory cytopenia with multilineage dysplasia
  • Refractory anemia with excess blasts
  • Myelodysplastic syndrome with isolated del(5q)
  • Myelodysplastic syndrome, unclassifiable

•Childhood myelodysplastic syndrome

Provisional entity: refractory cytopenia of childhood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What cells stand out as being wierd here?

A

Megakaryocytes with seperate nuclei - should have 16-32 fused nuclei normally

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What is outlined in this?

What is wrong with it?

A

Pelgeroid neutrophil (anytime we hear …oid, such as this, or megaloblastoid, we should be thinking myelodysplasia)

Has only two lobes instead of 3-4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What is outlined here?

A

A nucleated RBC precursor with multiple nuclei (budding nuclei)

50
Q

What is shown here?

Stain?

A

Ringed sideroblasts - iron laden mitochondria

Prussian blue stain

51
Q

What characterizes the blood and bone marrow cellularity in refracroty cytopenia with unilineage dysplasia?

A

Blood - cytopenia

Bone marrow - dysplasia (>10%) in one lineage only

52
Q

What characterizes the blood and bone marrow finding in refractory anemia with ring sideroblasts?

A

Blood - anemia, no blasts

Marrow - Erythroid dysplasia only

>15% ringed sideroblasts

53
Q

What characterizes refractory cytopenia with multilineage dysplasia?

A

Blood - cytopenias, monocytes w/o auer rods

Marrow - Dysplasia in >10% of the cells of 2 or more myeloid lineages

54
Q

What are the types of refractory anemia with excess blasts?

A

RAEB 1

RAEB 2

55
Q

What characterizes the blood and bone marrow findings in RAEB 1?

A

Blood - <5% blasts and no Auer rods

Marrow - 5 - 9% blasts, no Auer rods

56
Q

What characterizes the blood and bone marrow findings for RAEB 2?

A

Blood - 5 - 19% blasts, Auer rods possible

Marrow - 10 - 19% blasts, Auer rods possible

57
Q

What characterizes the blood and bone marrow findings in myelodyspastic syndrome with isolated del(5q)?

A

Blood: anemia, platelets unchanged or increased, no auer rods

Marrow: ↔ or ↑ megakaryocytes with hypolobated nuclei

58
Q

MDS

Affects individuals older than 50 years (mean age of onset 70 years), presents with weakness, infection and hemorrhage caused by bone marrow failure and peripheral pancytopenia.

~ 50% discovered incidentally during routine blood testing for another reason.

If it looks like If it looks like myelodysplasia and has >1000 monocytes/uL in the blood then what does the patient have?

A

Chronic myelomonocytic leukemia

59
Q

If it looks like myelodysplasia or a myeloproliferative syndrome and there is more than 20% blasts in the bone marrow or blood then the patient has?

A

Acute myeloid leukemia

60
Q

MDS has come good prognostic groups that may live 5 or more years. What is the median survival in primary MDS?

A

9 - 29 months

61
Q

MDS is serious in its own right without progression to AML. What are the odds of progression to AML?

Outlook once this happens?

A

10 - 40%

Prognosis is dismal, at 6 or fewer months survival

62
Q

What is one strategy to maintain a patient with MDS?

A

Regular transfusions

63
Q

In chronic myeloproliferative disorders, how is the terminal differentiation of the neoplastic clone affected? Where would you find mature end-stage cells to be increased?

What is this type of condition associated with?

A

•The terminal differentiation of the neoplastic clone is unaffected and mature end-stage cells are increased in the peripheral blood in markedly increased numbers

Associated with an abnormal increase in the activity of mutated tyrosine kinases

64
Q

If I say chronic myelogenous leukemia then you say the mutation associated is?????

A

BCR-ABL fusion gene

65
Q

What is the consequence of the mutation in CML?

A

BCR-ABL fusion gene causes constitutive ABL kinase activation

66
Q

What mutation is found in over 95% of polycythemia vera cases?

A

JAK2 point mutations

67
Q

What does the mutation in polycythemia vera cause?

A

Constitutive JAK2 kinase activation

68
Q

What mutations are associated with essential thrombocytopenia?

Consequences?

A

JAK2 - 50 - 60%

MPL 5-10%

constitutive activation of JAK2 and MPL respectively

69
Q

What are the mutations associated with primary myelofibrosis?

Consequences?

A

JAK2 50-60%

MPL 5-10%

constitutive activation of either JAK2 or MPL

70
Q

What are the myeloproliferative neoplasms?

A

• Chronic myelogenous leukemia, BCR-ABL1–positive

  • Chronic neutrophilic leukemia
  • Polycythemia vera
  • Primary myelofibrosis
  • Essential thrombocythemia
  • Chronic eosinophilic leukemia, not otherwise specified
  • Mastocytosis
  • Myeloproliferative neoplasms, unclassifiable
71
Q

What distinguishes CML from other chronic MPDs?

A

Presence of a chimeric BCR-ABL gene derived from portions of BCR on 22 and ABL on 9. present in more than 90% of cases.

72
Q

What do we can the (9;22)(q34;q11) mutation that causes the BCR-ABL fusion gene?

A

Philadelphia Chromosome (Ph)

73
Q

Name all the cancers the philadelphia chromosome is found in. (that we covered in this course anyway)

A
  1. CML
  2. ALL sometimes
  3. AML sometimes
74
Q

CML presents with increased precursors of all cell lines including megakaryocytes, peripheral marked leukocytosis 50,000 or even 100,000 total granulocyte count with PMNs, bands, metamyelocytes, myelocytes.

What else is/may be significant about the peripheral blood?

What will be notable about the marrow?

A

Blood - may see basophilia, less than 10% blasts (otherwise acute)

Marrow - Sea-blue histiocytes may be present. Marrow will be 100% cellular (no fat)

75
Q

What tends to mimic CML?

What are some ways to differentiate?

A

Leukamoid reaction

  1. Alkaline phosphatase score (low in CML)
  2. Look for Ph chromasome
  3. Flow cytometry
76
Q

BCR-ABL activates multiple downstream pathways, which drive growth factor-independent proliferation and survival of bone marrow progenitors.

What is the impact on differentiation and the net result on the cell population in peripheral blood?

A

BCR-ABL does not interfere with differentiation, the net result is an increase in mature elements in the peripheral blood, particularly granulocytes and platelets.

77
Q

If you are presented with the attached karyotype, what is it most likely to be associated with?

A

CML

78
Q

What is a useful adjuct detection method for BCR-ABL, JAK or MPL mutations?

A

FISH

79
Q

The natural history of CML is one of slow progression with moderate anemia and hypermetabolism (high cell turnover).

What are the main symptoms/findings?

A

weakness

fatigue

weight loss

enlarged spleen (markedly)

80
Q

What can the massive splenomegaly in CML lead to?

A

Splenic infarcts

81
Q

The median survival in CML w/o treatment is 3 years. there are two primary progressions that occur. What occurs in each and what percent go down each path?

A
  • 50% of patients enter an “accelerated” phase (worse anemia and thrombocytopenia) then enter after 6 to 12 months a “blast crisis”—-development of acute leukemia
  • Other 50% eventually develop “blast crisis” without accelerated phase
82
Q

What do 70% of CML patients develop?

What do 30% of CML patients develop?

A

•70% develop myeloid leukemia and 30% pre-B leukemia

83
Q

What is the Rx for CML?

A

Rx- Gleevac/imatinib (BCR-ABL kinase inhibitor)

84
Q

What is the likely cell of origin in CML?

A

Multipotent progenitor

85
Q

Where does CML typically hit the spleen?

A

The red pulp

86
Q

What is the median age at dx for CML?

A

64

87
Q

What is the mean age at death of CML patients?

A

76

88
Q

What cellularity will you see with true polycythemia?

Peripheral blood hematocrit will be roughyl 60%. What can the increases in red cell mass produce?

A

Panmyelosis, with or without basophilia

Increased RBC mass produces symptoms related to hyperviscosity syndromes with thromboses and infarcts.

89
Q

In polycythemia vera erythroblasts are hypersensitive to EPO, why?

What are serum EPO levels like?

A

D/t JAK2 V617F mutation

serum EPO suppressed

90
Q

PV has a median age of onset of 60 years and presents as plethoric and cyanotic. Why in a codition with shitloads of RBCs do we have cyanosis?

A

Hyperviscosity reduces flow rate through the lungs, which of itself reduces the volume of RBC’s being oxygenated.

Further, the RBC’s move slower through the tissues, and thus more O2 is stripped offr as they transit.

The result is decreased O2 delivery to all tissues.

91
Q

What are some other elements of the clinical picture of a PV patient?

A
  1. bleeding and thrombosis d/t elevated but abnormal platelets
  2. massive splenomegaly
  3. pruritis and peptic ulcers d/t released histamine from increased basophils
  4. 5-10% develop gout (hyperuricemia d/t nuclei breakdown)
92
Q

PV typically presents with Hb 14-28 gm/dL and Hct over 60%, WBC 12,000-50,000/mm^3 and platelets over 500,000/mm^3.

What can be done to control the symptoms and complications?

What is the untreated survival?

With extended survival by tx, what do 15-20% tend to evolve?

A

Phlebotomy - blood letting

Months if untreated

15-20% evolve a “spent phase” during which clinical and anatomic features of primary myelofibrosis develop.

93
Q

What besides phebotomy can be done for PV?

Possible complications?

A

Chemo is also an option, but this increases the odds of developing acute leukemia.

AML can develop in 1-2% of PV patients anyway

94
Q

Essential thrombosis is a diagnosis made through exclusion of all else. What mutations are common here?

Describe the levels of polycythemia and speed of progressive marrow fibrosis?

A

JAK2 (50%) and MPL (5-10%)

No polycythemia or progressive marrow fibrosis

95
Q

Essential thrombosis has what bone marrow cellularity changes?

A

•Bone marrow cellularity only mildly to moderately increased but megakaryocytes are substantially increased in number

96
Q

PAtients with essential thrombocytosis see thromboses, bleeding and erythromelalgia occur during the diseases indolant course after onset at ~60 years of age.

What hell is erythromelalgia?

A

Erythromelalgia is a rare condition that primarily affects the feet and, less commonly, the hands (extremities). It is characterized by intense, burning pain of affected extremities, severe redness (erythema), and increased skin temperature that may be episodic or almost continuous in nature.

97
Q

What do you see here?

What is this associated with?

A

Giant Platelet - platlet equal in size to RBC

Essential thrombocytosis

98
Q

What do you see here, in this patient’s bone marrow sample? The patient has essential thrombocytosis fyi.

A

Numerous megakaryocytes which are larger than normal and have significantly less nuclear lobulation than normal.

99
Q

What is the hallmark of primary myelofibrosis?

A

rapidly developing obliterative marrow fibrosis caused by extensive collagen deposition by non-neoplastic fibroblasts

100
Q

What causes the non-neoplastic fibroblasts to run amok in primary myelofibrosis?

A

PDGF and TGF-β

101
Q

What is an eventual consequence of the fibrosis seen in primary myelofibrosis?

A

Obliteration of much of the marrow space and production of abnormal dysplastic precursor forms. (large clustered megakaryocytes) Cytopenias also ensue.

102
Q

What mutations are associated with primary myelofibrosis?

A

JAK2 (50-60%) and MPL (1-5%)

103
Q

Primary myelofibrosis has an average age of onset after 60 y/o with initial symptoms of progressive anemia or marked splenomegaly (with or without infarction), caused by extramedullary hematopoiesis. These patients may develop hyperuricemia and gout.

What will be seen in the peripheral smear?

A

leukoerythroblastosis

104
Q

What is the aggression level of primary myelofibrosis?

What do 5-20% of these cases develop?

A

Aggressive, median survival 3-5 years

5-20% may develop AML-like blast crisis

105
Q

At what point in the progression of primary myelofibrosis is this patient?

A

Late stage - if you hit this with trichrome stain you’d see tons of blue in the fibrosis.

106
Q

At what point in the progression of primary fibrosis is the patient this bone marrow sample was taken from?

A

Early - note the clustering of megakaryocytes

107
Q

What abnormalities do you see in this peripheral blood smear from a patient with primary myelofibrosis?

A

Dacrocytes - tear drop shaped RBCs

Two nucleated erythroid precursors (not a good sign)

While not unique for primary myelofibrosis, represents some of the findings possible in a peripheral blood smear.

108
Q

LAngerhans cell histiocytosis is also known as what?

A

Eosiniphilic granuloma

109
Q

Describe the immature dendritic cells seen in langerhans cell histiocytosis.

A

Vesicular nuclei with linear grooves or folds

On EM has “Birbeck” granules (HX bodies) composed of pentalaminar tubules with dilations

110
Q

What are the cell surface markers that are important regarding langerhans cell histiocytosis?

A
  1. S-100+
  2. CD1+
  3. CD207+ (langerin)
  4. CD1a+
  5. HLA-DR+
111
Q

The Langerhans cells in eosinophilic granuloma express CCR6, like in normal langerhans cells (CCL20 ligand in skin and bone).

What do they express abnormally?

A

Express CCR7 abnormally (CCL19 & CCL20 in lymphoid tissues)

112
Q

Where does langerhans cell histiocytosis love to go to?

A

The skin

113
Q

Most cases of langerhans cell histiocytosis occur in childhood, with an incidence of 5/1 million per year. It impacts caucasions much more often than blacks.

Two types that can be seen are unifocal and multisystem disease. What are their respective prognoses?

A

Unifocal disease (>99% 5-year survival)

Multisystem disease (66% mortality)

114
Q

Which cell surface markers are “specific” and “pretty specific” for Eosinophilic granuloma?

A

Specific: CD207+ (langerin)

Pretty specific: CD1a+

115
Q

What is Letterer-Siwe disease?

A

Multifocal multisystem langerhans cell histiocytosis

116
Q

Letterer-Siwe disease most often presents before age 2, and with chemo we see 50% 5 year survival. The untreated disease is rapidly fatal.

What is the clinical presentation?

A
  1. Cutaneous lesions resembling a seborrheic eruption (langerhans cells infiltrate)
  2. hepatosplenomegaly
  3. lymphadenopathy
  4. pulmonary lesions
  5. bone lesions
117
Q

What are the four different presentations we see with langerhan’s cell histiocytosis?

A

Unifocal lesions

Multifocal unisystem

Multifocal multisystem

Multifocal unisystem (pulmonary)

118
Q

Unifocal lesions occur in older children or adults, where do they typically occur?

A
  • Skeletal system
  • Also…
    • skin
    • lung
    • stomach
119
Q

Multifocal unisystem langerhans cell histiocytosis will have multiple erosive bony masses in young children.

What will 50% of these kids develop?

A

Diabetes insipidus d/t involvement of the posterior pituitary stalk of the hypothalamus

120
Q

What triad is associated with multifocal unisystem langerhans cell histiocytosis?

A

Hand-Schiuller-Christian Triad

  1. Calvarial bone defects
  2. diabetes insipidus
  3. exopthalmos
121
Q

Many patients with langerhans cell histiocytosis experience spontaneous regression. What are the treatment options available?

A

Chemotherapy if multifocal

Local excision or irradiation if unifocal

122
Q

What are these? Describe them.

A

Birbeck granules

Trilaminar rod structures with focal dilatation

Heme/Lymph - Exam 1 (5 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions