Diseases of White Blood Cells and Lymph Nodes Flashcards Preview

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

Flashcards in Diseases of White Blood Cells and Lymph Nodes Deck (69):
1

What is neutropenia?

Neutrophil count < 2 x 10^3 / uL

(Normal = 3.2 - 6.2 x 10^3)

2

What is agranulocytosis?

Nuetrophil count < 1 x 10^3 / uL

(Normal = 3.2 - 6.2 x 10^3)

3

The bone marrow is hypercellular in which causes of neutropenia?

  • Increased destruction
  • Ineffective granulopoiesis

4

The bone marrow is hypocellular in which causes of neutropenia?

  • Conditions that suppress or destroy granulocyte precursors

5

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

  • Neutrophils are being consumed in the periphery

6

What is lymphocytopenia?

Lymphocyte count < 1.5 x 10^3 uL

(Normal = 1.5 - 3 x 10^3)

7

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

Protein - calorie malnutrition

8

What is leukocytosis?

Total leukocyte count > 11 x 10^3

 

9

What is a leukemoid reaction?

Leukocyte count =  25 - 30 x 10^3

Normal response to cytokines

10

What are the common causes of neutrophilia?

  • 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

11

What is Eosinophilia?  When does this occur?

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

12

What is Monocytosis?  What does this result from?

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

13

What is Lymphocytosis?  When does this occur?

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

14

What does Basophilia often result from?

Myeloproliferative disease

15

What are the characteristics of Acute Lymphadenitis?

  • 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

16

What are the characteristics of Chronic Lymphadenitis?

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

17

The majority of lymphoid neoplasms are what type of cell?

B cell (80%)

18

Which Lymphomas are classified as "Indolent?"  What is the prognosis?

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

19

Which lymphomas are classified as "aggressive?"  What is their prognosis?

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

20

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

  • Lymphomas
    • Burkitt's
    • Precursor B and T ALL
    • Adult T-cell 

21

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

Langerhans histiocytes

22

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

IL2 receptor alpha chain found on activated T and B cells

23

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

germinal center B cells

24

CD21 is a cell marker specific to which cell type?

Dendritic cells

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