Pathology 4,5,6 : White Blood Cell Pathology Flashcards Preview

Hematology-Oncology Exam 2 > Pathology 4,5,6 : White Blood Cell Pathology > Flashcards

Flashcards in Pathology 4,5,6 : White Blood Cell Pathology Deck (194):
1

Which is more prevalent in Acute WBC disease: Immature or Mature cells being produce ?

Immature, typically due to -blast expansion

In Chronic WBC disease you will see more mature cells being expanded,

2

What cells are typically seen in Lymphocytic Leukemia/Lymphomas ?

Lymphoid lineage cells: T and B cells.

3

What cells are typically seen in Myeloid Leukemia/Lymphomas ?

Myeloid lineage cells: Granulocytes/Monocyte (Neutrophil, Basophil, Eosinophil), Megakaryocytes (platelets).

4

What is leukopenia ?

Low levels of circulating WBCs

agranulocytosis (Neutrophils, Basophils and Eosinophils) often lead to fatal infections and sepsis !


5

What is often seen in conjunction with Leukopenia ?

anemia and thrombocytopenia ( especially if leukopenia is due to suppression of Common Myeloid Progenitor Cells)

6

Common causes of leukopenia are (4)...

Medications

Vitamin deficiencies

Malignancies ( Anything growing in the white bone marrow (malignancies) will cause leukopenia ).

Splenic sequestration (spleen can soak up WBC’s.)

7

Absolute Neutropenia (loss of neutrophils) is often caused by deficiencies in which vitamins ?

B12 /Folate --> Megaloblastic Anemia

8

Suppression of GRANULOCYTIC precursor cells ( -->Absolute Neutropenia and agranulocytosis) is often due to ...

Exposure to certain medications:

Chemotherapy
Chloramphenicol
Sulfa drugs
Chlorpormazine
Clozapine (Anti-psychotic) **** ON BOARDS.

9

Suppression of MYELOID STEM CELLS (-->Absolute Neutropenia and Agranulocytosis) is often the result of...

Aplastic Anemia, Leukemias, Malignant Lymphomas

10

Besides being caused by ineffective or suppressed neutrophil production, Absolute Neutropenia can be cut to ACCELERATED REMOVAL of neutrophils in which organ ?

Spleen (Splenic Sequestration )

Also, increased removal can be caused by:

-Septicemia
-Immunologic damage to Neutrophils (As seen in Felty Syndrome: rheumatoid arthritis, splenomegaly and neutropenia.)

11

What is leukocytosis ?

Increased number of WBCs in blood (the type of cell lineage expanded really determines severity)

12

What is the most common cause of Leukocytosis of Neutrophils ?

Infection of some varying degree ( Pyogenic Bacterial Infections, Sepsis etc.)

13

Leukocytosis of Eosinophils in the absence of asthma, allergies or parasitic infections should direct you toward which pathology ?

Malignancies

14

Active Pulmonary Tuberculosis, Brucellosis/Rickettsiosis, Malaria and Inflammatory Bowel Disease can all lead to Leukocytosis of which WBC lineage ?

Monocytes.

15

Sustained activation of the immune system by acute viral infections, brucellosis , active TB and Malignancy will lead to an expansion of what WBC lineage ?

Lymphocytes (T and B cells)

Focus mainly on Sustained Activation due to Viral Infections.

16

What is the main differential between Reactive Leukocytosis and Acute Leukemia ?

Both will have increased levels of Leukocytes, however Acute Leukemia will be distinguished by the presence of BLASTS in peripheral blood.

17

A form of non-neoplastic leukocytosis which mimics acute/chronic leukemia.....

Leukemoid Rxn

18

What are the three main causes of a leukemoid Rxn ?

-Chronic inflammation
-Chronic infection
-Medications (Steroids) --> Demargination and release into blood stream

19

Enlargement of lymph node(s) of any etiology...

Lymphadenopathy

20

What is lymphadenitis ?

Painful enlargement of lymph nodes

21

What is Acute lymphadenitis ?

occurs in nodes draining organs infected with bacteria.

22

What is Acute Non-Specific Lymphadentis ?

lymph nodes with reactive changes secondary to infections, toxic products or foreign matter from wounds (Not just bacteria as in Acute lymphadenitis).

23

Do lymphadenitis and lymphadenopathy lead to organized or disorganized nodal architecture ?

Organized. Well marginalized germinal centers.

24

Follicular Hyperplasia seen in Chronic Non-Specific Lymphadenitis leads to the activation of which kind of cells ?

B Lymphocytes.

25

Sinus Histiocytosis (a subset of Chronic Non-Specific Lymphadenitis) is characterized by distension of sinusoids in lymph nodes draining.....

Primary carcinomas.

26

What is Paracortical Lymphoid Hyperplasia (a subset of Chronic Non-Specific Lymphadenitis) ?

reactive changes in T-lymphocyte regions which transform into immunoblasts

Sometimes seen with vaccines and exposure to certain drugs (phenytoin)

27

Malignant neoplasms of LYMPH NODES composed of MALIGNANT LYMPHOID cells ( More mature B and T-cells) are characterized as ....

Lymphomas

(May also occur in lymphoid tissue outside of lymph nodes like the spleen)

28

Neoplasms of HEMATOPOIETIC immature cells that involves the MYELOID BONE MARROW are characterized as ...

Leukemia (also, Myeloproliferative diseases)

29

Localized or disseminated malignant proliferation of plasma cells or B-Cell lymphoid cells leading to the production of monoclonal Ab's are known as

Plasma Cell Dyscrasias

30

What is a histiocytoses ?

Neoplasms of histiocytes (monocytes)

31

What is the Cell Surface Marker associated with pluripotent hematopoietic stem cells ?

CD 34+

32

NK cells have which cell surface marker ?

CD 56+

33

What cells are over-produced and lack differentiation in Acute Myeloblastic Leukemia ?

Myeloblasts (will not differentiate correctly but will expand their own lineage )

34

What cells are often depleted in Acute Myeloblastic Leukemia ?

RBC and Platelets

35

Acute Lymphoblastic Leukemia occurs due to over-expansion of which cells ?

Lymphoblasts

36

What are the two main categories of Malignant Lymphomas ?

Hodgkins

Non- Hodgkins

37

Pathologic diagnosis of Lymphomas requires both
Standard Tissue Light Microscopy and
Immunophenotyping by either immunocytochemistry or Flow Cytometry (Looks for markers on the cells). Which type of biopsy is preferred, fine needle aspirate or excisional ?

Excisional (need to see tissue architecture)

38

Of the 5 categories based on morphology, genotype, clinical features, immunophenotyping for characterizing lymphomas, what are the four associated with Non-Hodgkins ?

-Pre-Cursor B-Cell Neoplasm (Immature B-Cell)
-Peripheral B-cell Neoplasms (mature B-cells)

-Precursor T-cell Neoplasms (immature T-cells)
-Peripheral T-cell and natural killer cell neoplasms (mature T-cells and NK cells)

39

The fifth category based on morphology, genotype, clinical features, immunophenotyping for characterizing lymphomas is Hodgkins Lymphoma. What is major diagnostic difference between Hodgkins and Non-Hodgkins Lymphoma's ?

Reed Sternburg Cells (CD30+)

40

On what cells will you see CD45 ?

ALL WBC's !

41

List the CD marker that are Primarily T-cell associated ?

CD1, 3, 4, 5, 8.

LOW CD MARKERS ARE T-Cell

42

List the CD marker that are Primary B-cell associated ?

CD 10 ,19, 20, 21, 23 and 79

43

What drug is used for targeting CD20 on B-Cells ?

Rituximab.

44

List the CD marker that are Primarily NK-cell associated ?

CD 16, 56

45

Plasma cell associated CD marker...

CD 138

46

B-cell Acute Lymphoblastic Lymphoma ( in nodes) or Leukemia (bone marrow) is a Low or High grade disease ?

HIGH (Must be treated immediately)

47

What does it mean for a disease to be Acute ?

It means that it is composed of PRECURSOR cells and will often show expansion of lympho/myeloBLASTS.

Look for these in biopsy .

48

In an Acute lymphoma/leukemia, what is a good marker to look for to determine is the lineage is Lymphoid or Myeloid ? (this will also confirm the disease is due to acute precursors rather than more mature chronic cells)

TdT+ (terminal deoxynucleotidyl transferase)

This will tell you the cell lineage is LYMPHOID and that it is immature.

49

What does a lymphoblast look like in microscopy

Large nucleus

very little cytoplasm

no granules seen (would indicate it's a granulocyte

lighter nucleus due to rapidly dividing chromatin.

50

What is the most common CATEGORY (5) of lymphoma ?

PERIPHERAL B-Cell Neoplasm (85% of all lymphomas)

Non-Hodgkins type.

There are many diseases that fall into this category (will be talked about later)...

-Small lymphocytic lymphoma/chronic lymphocytic leukemia
-Mantle Cell Lymphoma
-Follicular Lymphoma
-Hairy Cell Leukemia
-Diffuse Large B-Cell Lymphoma
-Burkitt’s Lymphoma

51

Small Lymphoctyic Lymphoma (a Peripheral B-Cell neoplasm ) is called what when leukocytes get greater than 4K lymphocytes ?

Chronic Lymphocytic Leukemia

52

What kind of cells are should you think of when you thing Small/Chronic Lymphocytic Leukemia ?

"Smudge Cells" ***

53

T- Cell Acute Lymphoblastic Lymphoma ( or Leukemia when bone marrow involved/circulating lymphoblast) is classified in which of the 5 major categories of lymphomas ?

Precursor T-Cell Lymphoma (you know it is precursor because it is an ACUTE lymphoma)

54

Is T-cell ACUTE lymphoblastic lymphoma a high or low grade disease ?

HIGH (Just like B-Cell ACUTE Lymphoblastic Lymphoma)

55

List some of the Peripheral T-Cell Lymphocytic Lymphomas (leukemias)

T

56

What are the 4 types (out of 5) of HODGKINS lymphoma that are associated with CD30* ?

Nodular sclerosis
Mixed cellularity
Lymphocyte-rich
Lympocyte depletion

57

What is the only type of HODGKINS lymphoma that is not associated with CD30 ?

Lymphocyte predominance

58

Non-Hodgkins Lymphomas (4 Classes) are distance from Hodgkins in that they do not express CD30+. On clinical exam you may find what kind of lymphadenopathy ?

rubbery, painless lymphadenopathy

59

Non-Hodgkins Lymphomas also present with "B-Type Symptoms". What are these ?

fevers, chills, night sweats

These are due to proliferation of the lymphocytes

60

Non-Hodgkins Lymphoma will affect which body areas (depending on disease of course)

Various Lymph Node groups (Typical Spots)
Oropharyngeal Region (Waldeyer’s Ring)
GI Tract
Bone Marrow
Skin

61

Involvement of which organ may lead to malignant lymphoid cells to appearing in peripheral blood ?

Bone Marrow
If you start to see large amount of B/T-cells in the blood, they will likely have lymphoma (despite this being the presentation for leukemia ?), look out for blasts

62

What are the two variables that must be accounted for when trying gain a pathologic diagnosis from a lymph node (excisional biopsy )

Cell Type (B-cell, T-cell)

Pattern of Growth : Follicular (Nodular) or Diffuse

63

Which typically has a better prognosis : Follicular (Nodular) or Diffuse ?

Follicular (Nodular)

Diffuse is much more difficult to treat.

64

Which of the 5 categories of Lymphoma does Follicular Lymphoma fall into ?

Peripheral B-Cell Neoplasms

65

What is the major chromosomal translocation associated with Follicular Lymphoma ?

**Chromosome 14:18 translocation !

66

What does the Chromosome 14:18 translocation in B-Cell with follicular lymphoma cause ?

The promoter for Ig Heavy chain is fused with the Bcl-2 gene leading to Bcl-2 overproduction

67

Increased Bcl-2 seen in follicular lymphoma will have what effect ?

Bcl-2 is an anti-apoptotic molecule thus, it will lead to enhanced survival of B-cells and increased expansion.

68

What kind of lymphadenopathy will you see in Follicular Lymphoma ?

Painless generalized LAD (Lymphadenopathy) found typically in only at Nodal Sites (Not GI, CNS, testes), may see in Spleen, though.


Shows a waxing and waining course

69

What is the treatment model for Follicular Lymphoma ?

Low dose chemo, only when the patient is SYMPTOMATIC (B symptoms, lymphadenopathy)

70

Are the germinal centers in Follicular Lymphoma organized or disorganized ?

Disorganized (in comparison to Reactive lymphadenitis and lymphadenopathy)

71

What is the most common FORM of Non-Hodgkin Lymphoma ?

Diffuse large B-cell lymphoma (A Peripheral B-Cell Neoplasm)

72

Is Diffuse large B-cell lymphoma a low or high grade ?

High Grade !

73

What is different about Diffuse large B-cell lymphoma from Follicular Lymphoma in respect to its site of dissemination ?

Can arise ANYWHERE in the body,

CAN INVADE THE CNS**

74

Heterogeneous tumor markers for Diffuse Large B Cell Lymphoma include

Bcl-6, Bcl-2 and c-myc

75

What category of lymphoma is Burkitts Lymphoma ?

Peripheral B-cell Neoplasm. (A tumor of mature B-Cells)

76

What two disease are associated with Burkitt Lymphoma ?

EBV
Malaria
HIV

Typically a co-infection of EBV with one of the other two (more likely malaria)

77

What is the path-mnemonic term for biopsy of someone with Burkitt Lymphoma and what causes it ?

**Starry sky pattern**

Macrophages ingesting apoptotic cells

78

Is Burkitt Lymphoma treatable ?

VERY MUCH SO ! Needs to be down quickly however.

79

What is the MOST COMMON translocation associated with B-cells

8:14

80

What occurs in the translocation of 8:14 in Burkitt Lymphoma ?

The Ig heavy chain promoters is fused to c-myc !

81

What is the result of over-expression of c-myc ?

c-myc signals for proliferation while simultaneously stopping the apoptotic actions of bcd-2
(yes, i know. Bcl-2 is anti-apototic and over-expressed in Follicular Lymphoma (14:18). It actually has a dual role, c-myc just turns off its apoptotic tendencies)

82

What Category of Lymphoma does Mantle-zone Lymphoma fall into ?

Peripheral B-Cell Neoplasm (NHL)

83

What is the translocation associated with Mantle-zone Lymphoma ?

11:14

84

What occurs due to the 11:14 translocation ?

Ig Heavy Chain promoter is fused to the gene for Cyclin D leading to overproduction

85

What does overproduction of Cyclin D lead to in Mantle-zone Lymphoma ?

Cyclin D promotes the G--> S phase transition in the cell cycles causing mitosis to occur more readily --> large numbers of B-Cells.

86

Describe the germinal centers seen in Mantle-zone Lymphoma

Atrophic germinal center with homogeneous population of small lymphoid cells

87

Describe the cells seen in Mantle-zone Lymphoma

irregular nuclear outlines, condensed chromatin, scant cytoplasm

88

What do cells that over-express Cyclin D look like on immunostain ?

Brown

89

How do you treat Mantle-zone Lymphoma ?

You Don't until it is symptomatic.

90

What Category of Lymphoma does Hairy Cell Leukemia fall into (yah, semantics are stupid on this one) ?

Peripheral B-Cell Neoplasm (NHL)

Chronic B-Cell Lymphoproliferative Disorder consisting of lymphoid cells in peripheral blood showing “hairy-like” cytoplasmic projections

91

What do Hairy Cell Leukemia cells stain positive for ?

TARTRATE RESISTANT ACID PHOSPHATASE (TRAP) !

92

Hairy Cell Leukemia is often seen in men with what co-morbidities ?

massive splenomegaly and pancytopenia

93

What are these 'hairy cells' seen in the blood despite being classified as a lymphoma ?

See them floating in the blood because the spleen is filled (splenomegaly)

94

What are the cell surface markers seen on Hairy Cells ?

The typical B-cell CD marker but also surface IgG, CD11c*, CD25*, CD103*

95

What is the treatment for Hairy Cell Leukemia

Cladribine (2-CDA; 2-chlorodeoxyadenosine

96

Mycosis Fungoides/Sezary Syndrome are in what category of Lymphoma ?

Peripheral T-Cell Neoplasm

97

Mycosis Fungoides/Sezary Syndrome T- cells Express CCR4, CCR10, CLA. What does this allow T-Cells to do ?

Home to the skin (don't really need to know CD markers)

98

What are the 3 phases of Mycosis Fungoides

Premycotic phase
Plaque phase
Tumor phase

99

What is the major differential between Mycosis Fungoides and Sezary Syndrome

In Sezary Syndrome, skin involvement is manifested as a generalized *exfoliative erytrhoderma*.

100

What is characteristic of Sezary Cells ?

cerebriform nuclei

Rarely progress to tumors

101

What are Pautrier’s micro abscesses ?

Seen in microscopy of Sezary Syndrome . They are really just the macrophages eating he cells.

102

In Hodgkins Lymphoma the cells produce CD30 ( except in Lymphocte Predominance HL). What is characteristic of the dissemination pattern in HL ?

Arise is a single node or chain of nodes and spreads in an anatomically contiguous fashion

103

Of the 5 forms of Hodgkins Lymphoma (Nodular Sclerosis ,Mixed Cellularity,Lymphocyte-rich,Lymphocyte Depletion ,Lymphocyte Predominance ) Which is most common ?

Nodular Sclerosis (70% of HL)

104

What age groups are most likely to get HL ?

People at age 30 and at age 60 (bimodal)

105

Reed Sternburg Cells have which cell surface markers ?

CD15+, ***CD30+***, Pax5+, CD45-

106

What kind of nucleus do Reed Sternburg Cells have ?

Bi-nucleate (these cells are though to arise from B-cells)
Mummification: shrinkage and pyknosis of malignant clone

107

Stage I HL :

-Involvement of single lymph node region (I) or
-Involvement of single extralymphatic lymph node site (Ie)

108

Stage II HL

Involvement of two or more LN on same side of diaphragm (i.e. Above or below the diaphragm.)

109

Stage III HL

Involvement of LN on both sides of diaphragm
(May include spleen (IIIs), or limited contiguous extralymphatic organ/tissue (IIIe, IIIes)

110

Stage IV HL

-Multiple or disseminated foci
-Involvement of one or more extralymphatic organs or tissues (with or without lymphatic involvement). If it grew from lymph into an organ its still stage III)

111

A or B staging ?

A = No symptoms
B= Symptoms (fever, Chills and Night Sweats)

112

What will you see in the lymph nodes in Nodular Sclerosing HL ?

Diffuse infiltrate of T-lymphocytes, eosinophils, macrophages and plasma cells with fibrous connective tissue bands (Very good prognosis)

Frequent Lacunar cells and occasional RS cells

Very "Ratty" looking lymph-nodes
NO EBV association

113

What virus is associated with Mixed Cellularity HL ? (2nd most common , don't worry too much about this.)

EBV

114

Which is the least common and yet most dangerous form of HL ?

Lymphocyte Depletion
Few lymphocytes but many RS cells
RS cells have classic phenotype (CD15+, CD30+, CD45-)

115

What patient are more prone to Lymphocyte Depletion HL ?

HIV+
Elderly

(there is also an association with EBV)

116

Lymphocte Predominance HL shows very few Reed Sturnberg cells but many Lymphocytes. What type of RS cells are often seen in this disease ?

Lymphohistiocytic RS cell variants (“popcorn cell”) within background of dendritic cells and B-lymphocytes (CD20+, BCL6+, CD15-, CD30-)



117

Is extra nodal presentation common in HL ?

NO it is very rare (much more common in NHL)

118

Plasma Cell Dyscrasias lead to the Neoplastic proliferation of plasma cell which produce Ig and Ig Fragments. What product can this lead to in the Urine ?

Bence-Jones Protein

119

What is the most common plasma cell dyscrasia ?

Monoclonal Gammopathy of Undetermined Significance

120

What are the respective urine M-Protein level and Bone Marrow Plasma Cell Concentrations seen in Monoclonal Gammopathy of Undetermined Significance ?

M protein level <10%

Differentiates MGUS from Multiple Myeloma (MGUS has a 1% chance of progressing to MM)

121

The neoplasm associated with Multiple Myeloma occurs where ?

In the bone marrow (Plasma Cell)

122

What are the respective urine M-Protein level and Bone Marrow Plasma Cell Concentrations seen in Multiple Myeloma ?

M protein levels >3 g/dL (Usually IgG, but sometimes IgA is produced)

Bone marrow plasma cells >10%

123

What are the two form of Multiple Myeloma ?

Smoldering and Symptomatic

124

What defines symptomatic MM ?

CRAB

Calcium- levels are elevated

Renal-Due to light chains in glomeruli

Anemia- (Due to overcrowding of bone marrow with plasma cells and bone destruction)

Bone Involvement- Lytic bone lesions from plasmactyomas --> Pathologic Fractures

125

When do you treat MM ?

In the symptomatic stage (CRAB)

Not really curable but many treatments

126

What causes the "Rouleux Formation" in RBC's in MM ?

IgG sticking to the RBC --> staking

127

What Ig is typically produced in Waldenstrom’s Macroglobulinemia ?

plasmacytoid B-lymphocytes producing only IgM** (MM is IgG typically)

128

Due to multimeric configuration, IgM in Waldenstrom’s Macroglobulinemia can lead to what syndrome ?

HYPERVISCOSITY SYNDROME -->Sluggish blood.
Visual changes and stroke are common

129

What do plasma cells secrete in Amyloidosis ?

Light Chain (either lambda or Kappa)

130

What other disease is Amyloidosis associated with ?

Multiple Myeloma

131

What is the stain used to see amyloid ?

CONGO RED

Also, shows apple green birefringence.

132

Where do you often see skin changes in patients with amyloidosis ?

Periorbital regions, tongue and extremities.

133

In leukemias, what is necessary to differentiate myeloid vs lymphoid lineage ?

Flow cytometry

134

Acute Leukemias occur due to neoplasm of hematopoeitic mature or immature cells ?

Immature

135

In the marrow , what will replace normal maturing cells in ALL or AML ?

BLASTS !

136

What type of cells are typically low in ALL or AML ?

Overall pancytopenia
Low neutrophils: infection,
Low platelets: bleeding
Low RBCs: anemia
These cell types are all depleted when these blasts are created

137

What are the characteristics of Acute Leukemias ?

Abrupt onset

Clinical Symptoms related to depression of normal bone marrow function (bleeding or infection)

Bone pain/tenderness

Leukemic blast infiltration of organs

CNS involvement (ALL>>>>>>>AML)

138

What demographic is mainly affected by Acute Lymphoblastic Leukemia ?

Predominantly a disease of children and young adults (boys, european descent)
Peak incidence: 4 years of age
80% of all childhood acute leukemias
Good Prognosis in kids (90% remission)

Very bad prognosis in adults

139

What are the characteristic signs of ALL ?

Bone Pain and Tenderness (filled w/ blasts)

Generalized Lymphadenopathy, Splenomegaly and Hepatomegaly: (more common in ALL than AML)


Central Nervous System Manifestations: Headache, vomiting and nerve palsies

140

Which translocation shows a better prognosis for children with ALL ?

12:21

141

What are the three factors associated with worse prognosis in ALL ?

Age < 2 (possible association with chromosome 11 translocations
ALL in adolescence or adulthood
Presence of t(9;22) Philadelphia chromosome

142

What demographic is mostly affected by Acute Mylelogenous Leukemia (AML)?

adults of middle/older age

143

What percentage of bone marrow must be Blasts to confirm a diagnosis of AML ?

20% blasts in bone marrow*… less than that is Myelodysplastic Syndrome.

144

WHAT IS THE CURE RATE OF AML (SORRY FOR CAPS)

Only 30% cure rate with treatment

145

What is the most treatable form of AML ?

M3: Promyelocytic (if you were to pick one, it would be this. Most treatable.)

146

M0

Minimally Differentiated AML (2-3%)

147

M1

AML without differentiation (20%)

148

M2

AML with maturation (30-40%) Most common form of AML.)

Auer Rods often Present

149

What translocation is often seen in M3 (promyelocytic) AML ?

(15;17) translocation to generate RARA/PML protein

150

What complication do patients with M3 AML often get ?

DIC

151

What is the treatment for M3 AML ?

All-trans retinoic acid (ATRA) and Arsenic Trioxide

152

M4

Acute Myelomonocytic Leukemia (15-20%)

shows both myelocytic and monocytic differentiation
+ non-specific esterase (Stain)

153

M5

Acute Monocytic Leukemia (10%)

associated with high incidence of organomegaly, lymphadenopathy and leukemic tissue infiltration

154

M6

Acute Erythroleukemia (5%)

megaloblastoid changes and some erythroid precursors with large or multiple nuclei

155

M7

Acute Megakaryocytic Leukemia (1%)

blasts of megakaryocytic lineage predominate
Reactive with platelet-related antibodies (GPIIb/IIIa or vWF)

156

What occurs to hematopoiesis in Myelodysplastic Syndromes.

Causes ineffective hematopoiesis (Maturation ARREST)

bone marrow partially or completely replaced by clonal progeny of abnormal stem cells

Leads to pancytopenia

157

What demographic usually gets Myelodysplastic Syndrome ?

Disease of older individuals (68 years median age)

158

What disease can Myelodysplastic Syndrome transform into ?

AML

159

What is the only real cure for MDS ?

Bone marrow Transplant

Can be treated with transfusion support and hypomethylating agents :
Decitabine
Azacitidine

160

What kind of patho-mnemonic cell types are seen in MDS ?

Pseudo Pelger-Huet Cell (Bi-lobed neutrophil)

161

Once again, what cells are path-mneomonic for Chronic Lymphocytic Leukemia/ Small Lymphocytic Lymphoma ?

SMUDGE CELLS

162

What is the Richter Transformation associated with CLL ?

CLL --> Diffust Large B-Cell Lymphoma (much more aggressive)

163

Hairy Cell Leukemia like Hairy Cell Lymphoma stain positive for....

Tartrate-Resistant Acid Phosphatase (TRAP)

164

Who do you sees HCL in ? What co-morbidities ?

Older Men

Pancytopenia, Splenomegaly.

165

What occurs in myeloproliferative diseases that leads to ineffective hematopoiesis ?

stem cell disorders showing MATURATION defects (Not arrest)

166

What disease (like MDS) can myeloproliferative (MPD) diseases transform to ?

AML (HIGH RATE OF TRANSFORMATION)

167

Polycytehmia Vera (MPD) occurs due to an overproduction of which cells ?

erythrocytes

168

What is the cause of Polycythemia Vera?

Jak2 mutation (95%)
Constantly on

169

What is a classic sign of Polycythemia Vera, aquagenic pruritus ?

Aquagenic pruritus – get itchy after taking a hot shower

170

What risk is increased by having Polycythemia Vera ?

CVA or VTE's

171

Essential Thrombocythemia is due to an over-expression of which cell type ?

megakaryocytes in bone marrow ---> Platelets in blood
Usually exceed 600,000 per mm3 with abnormally large platelets

172

What are symptoms associated with Essential Thrombocythemia ?

Thrombosis
Bleeding (weird backwards effect)

173

Chronic myeloid leukemia occurs because too many of these cells are created ...

Granulocytes (left-shifted neutrophilic precursors are produced)

174

What enzyme is abnormally low in Chronic myeloid leukemia ?

Alkaline Phosphatase

175

What translocation is commonly seen in Chronic myeloid leukemia ?

9:22 (this is the Philadelphia Chromosome)

176

What class of drugs are given to treat Chronic myeloid leukemia ?

BCR/ABL tyrosine kinase inhibitors

Imatinib (Gleevec)
Dasatinib (Tasigna)
Nilotinib (Sprycel)
Pomatinib (Iclusig

177

Myelofibrosis with Myeloid Metaplasia occurs due to a neoplastic transformation of which kind of cell ?

multipotent myeloid stem cell

178

What does the multipotent myeloid stem cell transformation cause to occur in the bone marrow space ?

deposition of collagen *

179

In Myelofibrosis with Myeloid Metaplasia you will see Maturing bone marrow cells outside bone marrow space in spleen, liver, lymph nodes. This is known as...

extramedullary hematopoiesis

usually see splenomegaly

180

What demographic typically gets Myelofibrosis with Myeloid Metaplasia ?

Usually occurs > 60 years of age or older

181

What stain can you use to visualize the collagen disposition in Myelofibrosis with Myeloid Metaplasia ?

Reticulin

182

What kind of appearance with RBC's have in patients with Myelofibrosis with Myeloid Metaplasia ?

Dacrocyte (Myelophthisic appearance) Getting Squeezed out

183

Histiocytosis is .....

massive expansion of macrophage line.

184

What can occur in malignant Histiocytosis ?

macrophages in the bone marrow that has become malignant. Fills in the compartmemtn – get anemia, thrombocytopenia, neutropenia

185

Red pulp of spleen

Area heavy with macrophages, lots of RBC sequestration and destruction.

186

White pulp of spleen

splenic lymphoid tissues

187

Acute Traumatic Rupture of Splenic Capsule requires ...

surgical repair/removal

188

What occurs in the thymus ?

T cell development, maturation, clonal selection

189

DiGeorge Syndrome is marked by Thymic Hypoplasia or Aplasia with absence of parathyroid gland. Deletion on this chromosome leads to this syndrome

22

190

Which branchial structures show impaired development in DiGeorge syndrome ?

of 3rd/4th branchial pouches

191

CATCH 22 (Di George)

Cardiac anomalies
Abnormal facies
Thymic aplasia
Cleft palate
Hypoparathyroidsim with hypocalcemia

192

Benign or encapsulated Thymoma is associated with...

aplastic anemia and PNH (paroxysmal nocturnal hematuria)

193

Type I Malignant Thymoma

Invasive Thymoma
cytologically benign but locally invasive

194

Type II Malignant Thymoma

Thymic Carcinoma
cytologically malignant