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Pathology > Diseases of White Blood Cells and Lymph Nodes > Flashcards

Diseases of White Blood Cells and Lymph Nodes Flashcards

1
Q

What is neutropenia?

A

Neutrophil count < 2 x 10^3 / uL

(Normal = 3.2 - 6.2 x 10^3)

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2
Q

What is agranulocytosis?

A

Nuetrophil count < 1 x 10^3 / uL

(Normal = 3.2 - 6.2 x 10^3)

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3
Q

The bone marrow is hypercellular in which causes of neutropenia?

A
  • Increased destruction
  • Ineffective granulopoiesis
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4
Q

The bone marrow is hypocellular in which causes of neutropenia?

A
  • Conditions that suppress or destroy granulocyte precursors
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5
Q

What does the presence of increased band neutrophils in the periphery indicate?

A
  • Neutrophils are being consumed in the periphery
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6
Q

What is lymphocytopenia?

A

Lymphocyte count < 1.5 x 10^3 uL

(Normal = 1.5 - 3 x 10^3)

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7
Q

What is the most common cause of reduced lymphocyte production worldwide?

A

Protein - calorie malnutrition

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8
Q

What is leukocytosis?

A

Total leukocyte count > 11 x 10^3

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9
Q

What is a leukemoid reaction?

A

Leukocyte count = 25 - 30 x 10^3

Normal response to cytokines

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10
Q

What are the common causes of neutrophilia?

A
  • Increased release from marrow stores
    • response to infx, inflammation, hypoxia
  • Demargination
    • inflammation, exercise, epinephrine, steroids
  • Decreased extravasation
    • steroids
  • Increased production of precursors
    • chronic infx, inflammation, cancer
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11
Q

What is Eosinophilia? When does this occur?

A
  • Eosinophilia
    • Counts > 400/uL
  • Causes
    • Type I hypersensitivity
    • Parasite
    • Drug rxn
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12
Q

What is Monocytosis? What does this result from?

A
  • Monocytosis
    • Counts > 800/uL
  • Cause
    • Chronic infection
    • Intracellular pathogens
    • Inflammatory bowel diseases
      • unique to monocytosis
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13
Q

What is Lymphocytosis? When does this occur?

A
  • Lymphocytosis
    • Counts > 5000/uL
  • Causes
    • Chronic infections
    • Intracellular pathogens
    • Pertussis
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14
Q

What does Basophilia often result from?

A

Myeloproliferative disease

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15
Q

What are the characteristics of Acute Lymphadenitis?

A
  • Direct infx of reticuloendothelial tissue or of material that entered the lymph
  • Nodes swell
    • Hyperplasia in germinal centers
    • Painful from capsule distension
  • Infx can result in necrosis
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16
Q

What are the characteristics of Chronic Lymphadenitis?

A
  • Associated w/ B-cell response
    • Follicular hyperplasia
  • Associated w/ T-cell response
    • Paracortical hyperplasia
  • Both
    • Swollen nodes
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17
Q

The majority of lymphoid neoplasms are what type of cell?

A

B cell (80%)

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18
Q

Which Lymphomas are classified as “Indolent?” What is the prognosis?

A
  • Lymphomas:
    • Follicular lymphoma
    • Small lymphocytic lymphoma
    • Mantle cell lymphoma
    • Marginal zone lymphoma
  • Prognosis
    • Not curable
    • Survival measured in years
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19
Q

Which lymphomas are classified as “aggressive?” What is their prognosis?

A
  • Lymphomas:
    • Diffuse large B-cell
    • Peripheral T-cell
    • Anaplastic large cell
  • Prognosis
    • some are curable
    • Survival of untreated disease measured in months
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20
Q

Which lymphomas are classified as “highly aggressive?” What is the prognosis?

A
  • Lymphomas
    • Burkitt’s
    • Precursor B and T ALL
    • Adult T-cell
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21
Q

CD 1 is a cell marker specific to which cell type?

A

Langerhans histiocytes

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22
Q

CD 25 is a cell marker specific to which cell type?

A

IL2 receptor alpha chain found on activated T and B cells

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23
Q

CD 10 is a cell marker specific to which cell type?

A

germinal center B cells

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24
Q

CD21 is a cell marker specific to which cell type?

A

Dendritic cells

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25
CD 79a is a cell marker specific to which cell type?
IgA = part of Ag receptor on mature B cells
26
CD 11c is a cell marker specific to which cell type?
Hairy cell leukemias
27
CD 15 and CD30 are cell markers specific to which cell type?
Reed Sternberg cells
28
CD 34 is a cell marker specific to which cell type?
Stem cells
29
What is the leukocyte common Ag?
CD45
30
Precursor B and T ALL * Cell morphology * Clinical Manifestations * Prognosis
* Cell morphology * Large nuclei * Scant cytoplasm * Pre-B * CD10, 19, 22, and TdT positive * Late also have mu chains * Pre-T * CD3, 7, and TdT * Clinical Manifestations and Path * Blast cells invade bone marrow * Myelosuppression (anemia, etc) * Fatigue * Fever * Bleeding * Bone pain (from marrow expansion) * Pre-T ALL * Thymus involved * Normally presents as a solid tumor * Prognosis * Bad: * t(9;22) aka Philadelphia chromosome * Good: * Hyperploidy * t(12;21)
31
Which form of ALL is most common?
Precursor B-cell type
32
Aggregation of Pro-lymphocytes (mitotically active) into proliferation centers within lymph nodes is pathognomonic for which white cell cancer(s)?
Chronic Lymphoblastic Leukemia (CLL) / Small Lymphocytic Lymphoma (SLL)
33
CLL and SLL are identical except for what characteristic?
Where lymphocytes are located! * CLL * Blood and marrow * SLL * Lymph nodes and other tissues
34
Chronic Lymphocytic Leukemia (CLL) and Small Lymphocytic Lymphoma (SLL) * Characteristics * Cell morphology * Clinical manifestations * Prognosis
* Characteristics * Peripheral B cell neoplasm * **Aggregation of pro-lymphocytes into proliferation centers** is pathognomonic * Neoplasias are **identical except lymphocyte location** * CLL: blood and marrow * SLL: Lymph nodes and other tissue * Cell morphology * **Smudge cells** * CD19, 20, 23, and 5 (a T cell marker) * Clinical manifestations * Nonspecific symptoms * Hypogammaglobulinemia * Prognosis * Variable, median 4-6 year survival * Deletions **11q** and **17p** = poor * **Prolymphocytic transformation** * worsening cytopenia * larger numbers of pro-lymphocytes * Diffuse large B-cell lymphoma (**Richter's Transformation**) * rapidly enlargening mass in a node or spleen * B cell hypertrophy
35
What is the Richter's transformation?
* In CLL/SLL * transformation of snormall small B cells to larger ones * Develop Diffuse large B-cell lymphoma
36
Follicular Lymphoma * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * Characteristic **nodular (follicular) pattern** of infiltration of tissue * (+) for sIg, CD 19, 20, and CD79a (CD5 is not expressed) * **Centrocytes** * irregular or cleaved nuclear contours, little cytoplasm * **Centroblasts** * Diffuse chromatin, multiple nucleoli * Lymphocytosis occurs in 10% * Path * t(14;18) * Over-expression of bcl-2 * Clinical Manifestations * Painless generalized lymphadenopathy * Uncommon: involvement of extranodal sites * Prognosis * Incurable * Survival 7-9 years (indolent) * Transformation to diffuse large B-cell lymphoma may occur
37
Diffuse Large B-cell Lymphoma * Cellular Morphology * Path * Clinical Manifestations
* Cellular Morphology * Large B cells with large nuclei * Multiple nucleoli * Abundant basophilic cytoplasm * Germinal Center Cells * CD19, 20, 22, 79a, sIg * Path * Overexpression of **bcl-6** (represses p53) * " bcl-2 or c-myc * Clinical Manifestations * rapidly enlarging, symptomatic, aggressive mass at a single nodal or extranodal site
38
Immunodeficiency-associated large B-cell lymphoma is associated with what infection?
latent EBV infx in HIV pts
39
Body cavity large cell lymphoma (Primary effusion lymphoma) is associated with what infection?
HHV-8 infx in HIV pts
40
Burkitt Lymphoma * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * Germinal center B cells * CD10, 19, 20, 22, 79a, sIg, and bcl-6 * "starry sky" cytoplasm = macrophages with ingested debris from apoptotic cells * Path * Translocation of c-myc gene on 8 * t(8;14) - by IgH chain * t(2;8) - by kappa light chain * t(8;22) - by lambda light chain * Clinical Manifestations * Sporadic form: rapidly expanding masses * Endemic BL: involves jaw, facial structures, visceral organs * Sporadic BL: intra- abdominal tumors * Prognosis * Most cured
41
Multiple Myeloma * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * Plasmacytoma (sheet-like mass) or diffuse * **Mott cell** * perinuclear clearning * Eccentric nucleus * Multiple blue vacuoles * sIg (-); **cIg (+)** * Path * B-cell clones secrete M component * Igs in serum (IgG, IgA) * L-chains in urine * Combines with Tamm Horsfall protein to form casts in renal tubules * Infiltrate bone marrow * Activate osteoclasts * vertebral column, ribs, skull most commonly affected * Clinical Manifestations * Bone pain * **"Punched out" bone lesions** * Hypercalcemia * Renal dysfunction (from L-chain proteinuria) * Uncommon: Hepatosplenomegaly, lymphadenopathy * Prognosis * Poor * May have spinal cord compression
42
What serum M component and urine L-chain component levels are diagnostic of Multiple myeloma?
* M component * \>3g/dL * L-chain * \>6g/dL
43
What is smoldering myeloma?
* Asymptomatic * Neoplastic plasma cells * 10-30% bone marrow cells * Serum M protein * \>3g/dL
44
What is Solitary Myeloma?
Solitary lesion associated with one site of infiltration Usually progresses to multiple myeloma over 10-20 years
45
Monoclonal Gammopathy of Uncertain Significance (MGUS)
* M protein in serum w/o symptoms * \<3g/dL * Same chromosomal aberrations as in Multiple myeloma * Very few progress to plasma cell dyscrasia
46
Lymphoplasmacytic Lymphoma * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * CD19, 20, 22, 79a, and surface IgM * No CD5 or CD23 (different from B-CLL * Path * **Dutcher body** (below) * Precipitated IgM * Intranuclear inclusion * **Russell bodies** * Cytoplasmic Ig * Secrete intact IgM * Causes **Waldenstrom macroglobulinemia** * (hyperviscosity syndrome) * Clinical Manifestations * Serum hyperviscosity * visual impairment * CNS symptoms * Bleeding * Cryoglobulinemia * Prognosis * Indolent
47
Mantle Cell Lymphoma * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * Resemble normal mantle B cells * CD5, 19, 20, 22 and sIg (IgM) * NO CD10 or 23 (dif. from CLL/SLL) * Absence of centroblasts / proliferation centers (dif from Follicular lymphoma) * Path * t(11;14) : over expression of cyclin D1 * promotes G1 to S * Clinical Manifestations * Painless lymphadenopathy * Splenomegaly * GI * Prognosis * Incurable
48
Splenic Marginal Zone Lymphoma * Cellular Morphology * Clinical Manifestations * Prognosis
* Cellular Morphology * Marginal zone B cell * Replace white pulp germinal centers * CD19, 20, 22, sIgM * NO CD5 or 10 * Clinical Manifestations * Slenomegaly * Lymphocytosis * Infiltrates marrow and liver * Prognosis * Indolent
49
Extranodal Marginal Zone Lymphoma * Cellular Morphology * Clinical Manifestations * Prognosis
* Cellular Morphology * CD19, 20, 22, 79a, sIgM * NO CD5, 10, 23 * Path * Invade MALT * Stomach most frequent site * Chronic inflammation * Prognosis * May subside if inflammation is resolved
50
Hairy Cell Leukemia * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * Characteristic **thin membrane protrusions** * Postgerminal center memory B cells * CD19, 20, 22, sIg (usually IgG), CD11c, CD25, and **CD103 (Sensitive marker for HCL)** * Path * Marrow Fibrosis * due to FGF and TGF-ß * Clinical Manifestations * Pancytopenia * Massive splenomegaly * Prognosis * Responds well to chemo
51
General Info: Peripheral T-cell Lymphomas * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * Highly pleomorphic * Reactive infiltrate of eosinophils and macrophages * Path * Destroy lymph node architecture * Clinical Manifestations * 7th decade * Generalized lymphadenopathy * Prognosis * Poor
52
Primary Cutaneous Anaplastic Large Cell Lymphoma * Cellular Morphology * Path * Prognosis
* Cellular Morphology * CD4, CD30 (a marker for Hodgkins Lymphoma) * (-) anaplastic lymphoma kinase * marker for primary systemic ALCL * Path * Infiltration of dermis and subcutaneous tissue only * Prognosis * Good
53
Primary systemic Anaplastic Large Cell Lymphoma * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * CD30, variable expression of T cell Ags * Path * t(2;5) = **Anaplastic lymphoma kinase** * Clinical Manifestations * Generalized lymphadenopathy * Skin involvement (1/4) * Rare involvement of marrow * Prognosis * Good
54
Adult T-cell Leukemia / Lymphoma * Cellular Morphology * Path * Clinical Manifestations * Prognosis
* Cellular Morphology * **HTLV-1 provirus positive** * CD2, 3, 5 * **Hyperlobated nuclei** * Path * Latent period 10-30 years * Clinical Manifestations * **Acute **onset: * lymphadenopathy * Hepatosplenomegaly * **Bone and cutaneous lesions** * Prognosis * survival 20 wks
55
Mycosis Fungoides aka: Cutaneous T cell Lymphoma * Cellular Morphology * Path * Clinical Manifestations
* Cellular Morphology * CD4, 2, 3, 45RO, 5 * Path * Infiltrating cells **form bands through tissue** * Clinical Manifestations: **Cutaneous** * Inflammatory preneoplastic phase * Resembles eczema * Plaque phase * Tumor phase * ulcerates
56
Sezary Syndrome * Cellular Morphology * Clinical Manifestations * Prognosis
* Cellular Morphology * CD4, 2, 3, 45RO, 5 * **Seazary cells** in peripheral blood * **cerebriform nucleus** * Clinical Manifestations * Erythroderma * Lymphadenopathy * Prognosis * Indolent
57
T cell variant Large Granular Lymphocytic Leukemia * Cellular Morphology * Clinical Manifestations
* Cellular Morphology * TCR, CD3, CD8 * Clinical Manifestations * Lymphocytosis * Neutropenia * Anemia
58
NK cell variant Large Granular Lymphocytic Leukemia * Cellular Morphology * Clinical Manifestations
* Cellular Morphology * CD56 * Clinical Manifestations * B symptoms (fever, weight loss, night sweats) * Hepatosplenomegaly * Anemia / Thrombocytopenia
59
Extranodal NK/T Cell lymphoma * Cellular Morphology * Path
* Cellular Morphology * Express NK markers * CD2 and **CD56** * Associated with **EBV** * Path * Invade small vessels * ischemic necrosis * Most commonly affects nose and sinuses
60
What are the clinical differences between Hodgkin and Non-Hodgkin Lymphomas? * Nodal involvement * Spread * Extranodal involvement
61
What are the characteristics of the four stages of Hodgkin and Non-Hodgkin Lymphomas?
* Stage I * Involvement of **single** lymph node region or single extralymphatic organ * Stage II * Involvement of 2 or more node regions on **same side of diaphragm** * Stage III * Involvement of lymph node regions on **both sides of the diaphragm** * Stage IV * Multiple or **disseminated** foci
62
Nodular sclerosis Hodgkins Lymphoma * Cell Morphology * Path * Prognosis
* Cellular Morphology * HRS Lacunar cells * CD15+, CD30+, EBV-, CD20- * Path * Bands of collagen divide tissue into nodules * Nodes affected: * Cervical * Supraclavicular * Mediastinal * Prognosis * Excellent * Presents in stage 1 or 2
63
Mixed cellularity Hodgkins Lymphoma * Cellular Morphology * Clinical Manifestations * Prognosis
* Cellular Morphology * Classic HRS and Mononuclear variants * CD15+, CD30+, EBV+ * Clinical Manifestations * Systemic * Night sweats * Weight loss * Prognosis * Presents in stage 3 or 4 * Poor
64
Lymphocyte rich Hodgkin's Lymphoma * Cellular Morphology * Path
* Cellular Morphology * Classic HRS cell and Mononuclear variants * CD15+, CD30+ * 40% EBV+ * Path * Infiltrate mostly T cells
65
Lymphocyte Depletion Hodgkins Lymphoma * Cellular Morphology * Clinical Manifestations * Prognosis
* Cellular Morphology * Reticular variant: * HRS cells (CD15+, CD30+, EBV+) * Very few inflammatory cells * Diffuse Fibrosis variant * **Hypocellular infiltrate** * CD15+, CD30+, EBV+ * **Non-collagenous fibrosis** * Clinical Manifestations * Diffuse fibrosis variant associated with systemic symptoms * Prognosis * Presents in stage 3 or 4 * Poor
66
Nodular Lymphocyte Predominant Hodgkins Lymphoma * Cellular Morphology * Path * Clinical Manifestations
* Cellular Morphology * **Popcorn cell** * CD20+ * Path * Atypical node involvement * **Inguinal** * Inflammatory infiltrate mostly B cells * Prognosis * Presents in stage 1 or 2
67
What is a potential complication of Lymphocyte Predominant Hodgkins Lymphoma?
Diffuse Large B-cell Lymphoma
68
Survivors of Hodgkin Lymphoma have an increased risk for which secondary cancers?
AML Lung cancer
69
What is the normal path of spread of Hodgkins Lymphoma?
Nodes =\> Spleen =\> Liver =\> Bone

Pathology (27 decks)

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