3.4b WBC Patholoy II Flashcards
(139 cards)
0
Q
What is the main difference between acute and chronic leukemia?
A
Number of blasts seen in PBS
1
Q
Malignant neoplasms of hematopoietic cells characterized by REPLACEMENT of BM by neoplastic cells, which eventually SPILL INTO BLOOD
A
Leukemia
2
Q
Subtypes of Leukemia (4)
A
- Chronic Lymphocytic Leukemia (CLL)
- Acute Lymphocytic Leukemia (ALL)
- Chronic Myelogenous Leukemia (CML)
- Acute Myelogenous Leukemia (AML)
3
Q
What disorders often accompany leukemia? (2)
A
- Thrombocytopenia
2. Anemia
4
Q
Lab test used to diagnose leukemia
A
Biopsy of BM
5
Q
- Most common cancer of children (F
3. Whites > Blacks
A
ALL
6
Q
Pathology in ALL
A
Mutation of genes required for B-cell development -> Blocked maturation
7
Q
Immunohistochemical marker for ALL: Immature cells (pre-B and pre-T lymphoblasts)
A
(+) Tdt
an immunomarker for immature cells :. all precursor cells would be Tdt+
8
Q
Immunohistochemical marker for ALL: Mature “late pre-B” ALL
A
(+) CD10, 19, 20
IgM heavy chain
9
Q
70% of T-ALLs have a mutation in?
A
NOTCH1, which is required in T-cell development
10
Q
In B-ALLs, there is mutation in? (4)
A
PAX5
E2A
EBF
t(12:21)
11
Q
Mass effect of ALL
A
Bone Pain
-Caused by neoplastic infiltration and marrow expansion of subperiosteum leading to bone resorption
Testicular enlargement
12
Q
Symptoms in ALL that are related to DEPRESSION OF MARROW FUNCTION (3)
A
- Fatigue d/t anemia
- Fever d/t infections secondary to neutropenia
- Bleeding d/t thrombocytopenia
13
Q
Complications seen in T-ALL are related to what?
A
Compression of larger vessels and airways in the mediastnum
14
Q
The ff indicate a favorable prognosis for which disease?
- 2-10 year olds
- Low white cell count
- Hyperploidy
- Trisomy of chromosomes 4,7,10
- Presence of a t(12:21)
A
ALL
15
Q
Low grade chronic B cell leukemia characterized by the presence of fine hair-like projections on the lymphocytes
A
Hairy Cell Leukemia
16
Q
What is the basis of leukemia in Hairy Cell Leukemia?
A
Proliferation of lymphocytes in the BM (decreased WBC in peripheral blood)
17
Q
Important to know about Hairy Cell Leukemia (3)
A
Massive splenomegaly
(+) TRAP
Middle-aged
18
Q
Pathology in Hairy Cell Leukemia
A
Proliferation of WBC (particularly lymphocytes) in BM enmeshed in a network of reticulum -> hard to pass through peripheral blood -> cytopenia -> splenomegaly
19
Q
Hairy cell leukemia typically expresses which markers? (3)
A
- Pan B-cell markers: CD19,20
2. Surface Ig, usually IgG
20
Q
Common manifestation in Hairly cell leukemis
A
massive splenomegaly
21
Q
What lab diagnosis is specific for hairy cell leukemia?
A
(+) TARP: Tartarate Resistant Acid Phosphatase
22
Q
Microscopic features of Hairy Cell Leukemia (2)
A
- Fried egg or honeycomb appearance
2. Fine hair-like projections on lymphocyte
23
Q
Which of the myeloid series:
- Contains large primary granules
- Nucleus is 50-60% of the cell size
A
Promyelocyte
24
Which of the myeloid series:
1. Sunset appearance (a clearing near the nucleus)
2. Nucleus is 50% of the size
3. First cell in the series to contain specific granules
Myelocyte
25
Which of the myeloid series:
1. Appearance with a slight indentation of the nucleus
2. Also with sunset appearance
Metamyelocyte
26
Which of the myeloid series:
| 1. Indentation reaches 50% of the nucleus
Stab cell
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Myeloid disorders (3)
1. Acute myelogenous leukemia (AML) - malignant
2. Myelodysplastic syndrome (MDS) - premalignant
3. Chronic Myeloproliferative Disorders (4)
28
In Chronic myeloproliferative disorders, there is the presence of all types of myeloid series (4)
Malignant
1. CML
2. Polycythemia vera
Premalignant
3. Essential Thrombocytosis
4. Myelofibrosis
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Myeloid disorder caused by the accumulation of immature myeloid forms in the BM
AML
30
Myeloid disorder where
1. stem cells are destroyed
2. ineffective hematopoiesis -> cytopenias
Myelodysplastic Syndrome (MDS)
31
Pathology in AML
Block in maturation of myeloid stem cell -> impede differentiation
32
Most cases of ALL are due to translocations between which two chromosomes?
Chromosome 12 and 21
33
DIC is unique for which classification of AML that are NOS (M0-M7)?
M3 (acute promyelocyte)
34
If both Auer rods (coalescent granules) and enzymes (myeloperoxidase) are present, it is classified under which classification of AML that are NOS?
M2 (mature)
35
What is the specific genetic abnormality seen in M3?
```
RARA gene (Retinoic Acid Receptor Alpha)
-located on chromosome 17
```
36
Absence of this gene leads to a block in the maturation of promyelocyte to myelocyte
RARA gene
37
Treatment for M3 (2)
1. Retinoid acid treatment
| 2. Chemotherapy
38
The ff are laboratory diagnoses for?
1. Elevated WBC counts
2. Low platelet and RBC count (d/t BM suppression)
3. >20% blast cells in WBC differential count
4. Special stains:
- Nonspecific esterase: monoblasts
- Myeloperoxidase: myeloblasts
- PAS: lymphoblasts
AML
39
Which classification of AML that are NOS is being described?
1. (+) myelocytes with les granules
2. Abundant cytoplasm
M2: AML with maturation
40
Which classification of AML that are NOS is being described?
1. (+) promyelocytes with Auer rods (look like dark scratches)
2. (+) primary granules in cytoplasm
M3: Acute promyelocyte
41
M0 or M5 (Myeloid without differentiation or acute monocytic leukemia) can be differentiated using what test?
Esterase Test
| -Monocytic cells are esterase (+) and have nuclei that are more irregular and cerebroid
42
Which classification of AML that are NOS is being described?
(+) RBC presursors
Royal blue cytoplasm
M6: Acute Erythroid Leukemia
43
What stain is used for cells of myeloid linease?
Peroxidase
| √positive result: fine brown dots in cytoplasm
44
Which stain is used for lymphoblasts?
PAS (Periodic Acid Schiff)
| √(+) result: large pink dots in cytoplasm
45
Which stain is used for monoblasts?
Nonspecific esterase
| √(+) result: red stain
46
Treatment for AML
BM transplant
47
s/sx for AML that are common for all forms (3)
1. Anemia
2. Decreased platelet
3. Neutropenia
48
(S/SX) What specific change can be seen in M3?
DIC
49
(S/SX) What specific change can be seen in M1 and M2?
Granulocytic sarcoma (chloroma) - tumurous infiltration of soft tissue and bones
50
(S/SX) What specific change can be seen in M4 and M5?
Infiltration of gums and skin (leukemic cutis)
51
In the case of granulopoiesis, the stage before the occurrence of anaplasia is?
```
Myelodysplastic Syndrome (MDS)
√myeloid stem cell is damaged
√can still produce cells but they're all abnormal -> maturation defects -> ineffective hematopoiesis
```
52
Pathology in Myelodysplastic Syndrome (MDS)
Stem cell damage -> maturation defects associated with ineffective hematopoiesis
53
Deletions and monosomy 5 &7 are seen in?
Myelodysplastic Syndrome (MDS)
54
Treatment of Myelodysplastic Syndrome (MDS)
BM transplant for younger patients
55
In which disease can the ff cellular abonrmalities that affect all nonlymhpoid cells be seen?
1. Ringed sideroblasts
2. Megaloblastoid maturation
3. Pawn ball megakaryocytes
4. Nuclear budding
5. Abnormal granulation
6. Pseudo Pelger-Huet cells
MDS
56
Disease in which a lot of different cells are produced but dies before entering peripheral blood
Myelodysplastic Syndrome (MDS)
57
What is the picture of the BM in MDS?
Hypercellular
58
PBS in MDS
Cytopenia
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In this disease, blast cells can be present. In inflammation, shift to the left occurs until myelocytes.
MDS
60
In this disease, the changes seen are so diverse that it affects RBCs, platelets, WBC, all of which results from STEM CELL DAMAGE :. it can lead to production of myelogenous leukemia.
MDS
61
In which disease can this be seen?
| +) Pseudo Pelger-Huet nuclei (2 lobes
MDS
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In ___, there is abnormal deposition of iron in the the perinuclear portion of the red cell (normally, iron must be evenly distributed)
MDS
63
It is the result of an increased production of one or more types of blood cells
Myeloproliferative Disorders (MPD)
64
Myeloprolifeative Disorders (4)
1. Chronic Myelogenous Leukemia
2. Polycythemia Vera
3. Essential Thrombocytosis
4. Myelofibrosis of BM
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It is the neoplastic transformation of multipotent progenitor cells capable of giving mostly to mature myelogenous cells
MPD
66
It is caused by a continuous stimulatory effect to multipotent or pluripotent progenitor cekks -> these cells lose normal proliferative control
MPD
67
In MPD, the abnormality is due to?
Mutated Tyrosine Kinase -> stem cell circumvents normal control -> growth factor-indepent proliferation and survival of marrow progenitors
68
Due to its mutation in MPD, this enzyme becomes "constitutively activated" but DOES NOT lead to impaired differentiation
Tyrosine Kinase
69
Normally, tyrosine kinase is activated by?
Hematopoietic growth factors
70
Variable transformation in MPD is characterized by? (2)
1. BM fibrosis
2. Peripheral blood cytopenias
Exhaustion from constant proliferation leads to a stop -> BM fibrosis -> cytopenia -> malignancy -> acute leukemia
71
Which disease involves the ff?
1. Involves large volumes of cells from the BM and
2. Homing of neoplastic cells from the BM to secondary hematopoietic organs (liver and spleen aka Extramedullary hematopoiesis) -> hepatosplenomegaly
MPD
72
What disease causes the ff pathology?
Causes a neoplastic transformation of pluripotent GRANULOCYTIC stem cells with NO BLOCK in maturation
Chronic myelogenous leukemia (CML)
73
What is the chromosomal abnormality in CML?
Translocation of ABL and BCR gene t(9:22) -> Philadelphia chromosome
74
Treatment of CML
Imatinib
75
Preferentially drive MEGAKARYOCYTIC progenitors and causes abnormal release of immature granulocytic forms from the BM to blood
CML
76
What enzyme is absent in WBC as seen in CML?
Alkaline phosphatase
√ kills bacteria
√ increased during infections
77
What is used to differentiate leukemoid reaction from CML?
Alkaline phosphatase
REMEMBER:
CML: (+) blast cells
Leukemoid reaction: (-) blast cells
78
PBS of?
1. Thrombocytosis
2. High platelet count
3. All stages of maturation are present
CML
*High platelet count indicates that it is chronic
79
Clinical features of?
1. First symptom
- dragging sensation in the abdomen d/t massive splenomegaly OR
- upper quadrant abdominal pain d/t splenic infarction
2. Hypermetabolism d/t increased cell turnover
3. Fatigability, weakness, weight loss, anorexia
4. Mild to medium anemia
CML
80
Stem cell disorder CONFINED to MEGAKARYOCYTE
Essential Thrombocytosis
81
What clinical characteristics seen in Essential Thrombocytosis are seen due to a large number of dysfunctional platelet? (2)
1. Thrombosis
| 2. Hemorrhage
82
PBS seen in?
1. Giant platelets with very high count
2. Mild leukocytosis
3. Modest degrees of extramedullary hematopoiesis -> organomegaly
Essential Thrombosis
83
Obliterative marrow fibrosis is the hallmark sign of?
Myelofibrosis: replacement of BM by fibrosis -> extensive extramedullary hematopoiesis -> hepato and splenomegaly
84
Its chief pathologic feature is: extensive deposition of collagen in the marrow by non-neoplastic fibroblasts
-> marrow failure
Myelofibrosis
85
Changes in the BM:
Hypercellular d/t increases in maturing cells of all lineages
-> becomes more hypocellular and diffusely fibrotic -> osteosclerosis (fibrotic marrow space is converted into bone)
Myelofibrosis
86
Sites of extramedullary hematopoiesis in Myelofibrosis (3)
spleen
liver
LNs
87
PBS of?
1. Leukoerythroblastosis
- d/t premature release of nucleated erythroid and early granulocytic progenitors
2. Dacrocytes (tear-drop shaped cells)
Myelofibrosis
88
Clinical characteristics of?
1. Progressive anemia: moderate to severe normochromic normocytic anemia
2. Splenomegaly
3. Abdominal enlargement
4. Hyperuricemia and gout
5. Fatigue, weight loss, night sweats
Myelofibrosis
89
Cells that results from B-cell proliferation that synthesize or secrete Ig or its fragments
Plasma cells
90
Plasma Cell Dyscrasias and Disorders (5)
1. Myeloma
2. Waldenstorm Macroglobulinemia
3. Heavy Chain Release
4. Primary/Immunocyte-associated amyloidosis
5. Monoclonal Gammopathy of Undetermined Significance
91
Benign counterpart of multiple myeloma
Plasmacytoma
92
What is the most common plasma cell gammopathy?
Multiple Myeloma
93
What protein is present in the urine of patients with Multiple Myeloma?
Bence Jones Protein
94
Pathology in MM? (2)
1. Increased M-protein
2. Multifocal destructive bone lesion throughout skeletal system
- affects central > long bones
95
What is seen in the xray of those with MM?
Soap bubble appearance
96
What is seen in the BM biopsy in MM? (3)
1. Replacement of marrow cells with plasma cells
2. Russel bodies (in cytoplasm)
3. Dutcher bodies (in nucleus)
97
Describe the morphology of normal plasma cells (2)
1. Clock-face nuclei
| 2. Eccentrically located nuclei
98
Clinical s/sx of?
1. Infiltration of bones
2. Recurrent infection d/t suppression of normal Ig
3. s/sx of renal insufficiency: Bence Jones Proteinuria
MM
99
Complications in MM (3)
1. Myeloma nephrosis
2. Myeloma nephropathy
3. Systemic amyloidosis
100
In the diagnosis of MM, what is seen in the urine sample? (1)
Bence Jones Protein, which is specific to MM
101
Rouleaux formation is present in __ d/t increased erythrocyte sedimentation rate
MM
102
In the lab diagnosis of MM, what is seen in blood or urine?
(+) M protein spike
103
Proliferation of IMMATURE DENDRITIC CELLS (Langerhans histiocytes)
Histiocytosis
104
The ff are microscopic characteristics of which cells seen in histiocytosis?
1. Folded/grooved nuclei
2. Moderately abundant pale cytoplasm, often vacuolated
3. Mixed with a few eosinophils
Langerhans cells
105
In histiocytosis, the proliferation of which cells is MONOCLONAL and is thus likely to be neoplastic in origin
Langerhans cell
106
Birbeck granules are characteristic of which disorder?
Histiocytosis
107
These are pentilaminar tubules often with a dilated terminal end producing a tennis racket-like appearance, which contain the protein langerin
Birbeck granules
108
It is also known as UNIFOCAL and multifocal unisystem Langerhans cell histiocytosis
Eosinophilic granuloma
109
Types of Histiocytosis (4)
1. Eosinophilic granuloma
2. Hand-Schuller-Christian Disease
3. Letterer-Siwe Syndrome
4. Pulmonary Langerhans Cell Histiocytosis
110
It is characterized by proliferation of langerhans cells admixed with variable numbers of EOSINOPHILS, lymphocytes, plasma cells, neutrophils
Eosinophilic granuloma
111
Where do eosinophilic granulomas usually arise?
Medullary cavities of bones
- calvarium
- ribs
- femur
112
In eosinophilic granuloma:
√ this most commonly affects the skeletal system in older children or adults
√ Indolent
√ may heal spontaneousl or cured by excision
Unifocal lesions
113
In eosinophilic granuloma:
√ usually affects young children
√ present with multiple erosive bony masses that sometimes expand to adjacent soft tissue
multifocal unisystem disease
114
It is aka Multifocal Unisystem Langerhans cell histiocytosis
Hand-Schuller-Christian Disease
115
Its symptoms include a combination of:
√ calvarial bone defects (skull)
√ diabetes insipidus
√ exophthalmos
Hand-Schuller-Christian Disease
note: the three symptoms is collectively known as the Hand-Schuller-Christian triad
116
What comprises the Hand-Schuller-Christian triad?
√ calvarial bone defects (skull)
√ diabetes insipidus
√ exophthalmos
117
If exophthalmos are present in adults, rather than in children, the disease is now known as?
Grave's disease
118
It is also known as Multifocal Multisystem Langerhans cell histiocytosis
Letterer-Siwe Syndrome
119
What is the dominant clinical feature in Letterer-Siwe Syndrome?
Development of cutaneous lesions that resemble a seborrheic eruption
120
Extensive infiltration of the marrow -> anemia, thrombocytopenia, predisposition to recurrent infections such as otitis media and mastoiditis
this is seen in?
Letterer-Siwe Syndrome
121
Who are affected in Letterer-Siwe Syndrome?
Infants
| Young children
122
The ff are symptoms of?
1. Fever
2. Concurrent hepatosplenomegaly, lymphadenopathy, pulmonary lesions, destructive osteolytic bone lesions (bone will have soft parts)
Letterer-Siwe Syndrome
123
This represents a special category of disease most often seen in adult smokers and usually comprises a polyclonal population of langerhans cells (which suggests that it is a reactive hyperplasia rather than a true neoplasm)
Pulmonary langerhans cell histiocytosis
124
It is a diversity of neoplasms that may arise from the thymus
Thymomas
125
Most thymomas arise from which region?
Superior mediastinum
126
Who are affected with thymomas?
Adults >40 years old. rare in children
127
What are thymomas?
Tumors of thymic epithelial cells
128
What are the (3) histologic subtypes of thymomas?
1. Benign, noninvasive
2. Benign, invasive/metastatic
3. Malignant
129
The ff are microscopic features of?
1. neoplastic epithelial cells arranged in a swirling pattern and have bland oval to elongated nuclei with inconspicuous nucleoli
2. only a few reactive lymphoid cells are interspersed
Benign thymoma (medullary type)
130
The ff are microscopic features of?
1. neoplastic epithelial cells are polygonal and have round to oval, bland nuclei with inconspicuous nucleoli
2. Numerous reactive lymphoid cells are interspersed
Malignant thymoma
131
The ff describe the morphology of?
1. lobulated, firm, white-gray masses
2. areas of cystic necrosis and calcification
3. emcapsulated
Thymoma
132
they are composed of medullary-type epithelial cells (Elongated or spindle-shaped) or a mixture of medullary- and cortical-type epithelial cells.
Noninvasive thymoma
133
There is a mixture of polygonal cortical-type epithelial cells and a denser infiltrate of thymocytes
Mixed thymoma
134
Refers to a tumor that is cytologically benign but locally invasive
Invasive thymoma
135
Macroscopically, they are usually fleshy, invasive masses, sometimes accompanied by metastases to sites like the lungs
thymic carcinoma
136
Microscopically, most are squamous cell carcinomas
Thymic carcinoma
137
a tumor composed of sheets of cells with distinct borders that bear a close histologic resemblance to nasopharyngeal carcinoma
Lymphoepithelioma-like carcinoma
138
Myasthenia gravis is a symptom of?
Thymomas
