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AP reviewer > Spleen > Flashcards

Spleen Flashcards

1
Q

Splenomegaly associated with systemic lupus erythematosus or with rheumatoid arthritis: Other findings (2).

A

Neutropenia.

Leg ulcers.

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2
Q

Reactive lymphoid hyperplasia without germinal centers: Cells (3).

A

Heterogeneous population of lymphocytes.

Immunoblasts.

Tingible-body macrophages.

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3
Q

Reactive lymphoid hyperplasia without germinal centers: Locations of infiltrate.

A

White pulp, including the periarteriolar lymphoid sheaths.

Infiltration of the trabeculae may lead to splenic rupture.

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4
Q

Castleman’s disease of the spleen: Most common type.

A

Plasma-cell type.

The hyaline-vascular type rarely or never occurs.

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5
Q

Castleman’s disease of the spleen: Types of multicentric disease.

A

Plasma-cell type.

Plasmablastic type.

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6
Q

Reactive follicular hyperplasia with formation of germinal centers: Gross pathology.

A

Nodularity of white pulp.

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7
Q

Castleman’s disease of the spleen: Histology of the plasma-cell type (2).

A

Hyperplastic or regressively transformed follicles.

Many plasma cells in the red pulp.

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8
Q

Castleman’s disease of the spleen: Histology of the plasmablastic type.

A

Fibrosis and many plasma cells surround hyperplastic or regressively transformed follicles.

Unremarkable red pulp.

Mantle zones contain immunoblastoid and plasmablastoid cells.

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9
Q

Castleman’s disease of the spleen: Microlymphoma.

A

Aggregates of HHV8-positive plasmablasts.

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10
Q

Castleman’s disease of the spleen: Immunophenotype of plasmablasts (4,1).

A

Positive: CD20, IgM, lambda light chain, HHV8.

Negative: CD30.

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11
Q

Castleman’s disease of the spleen: Laboratory finding associated with multicentric disease.

A

Increased IL-6 in the serum.

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12
Q

Castleman’s disease of the spleen: Complication of the plasmablastic type.

A

Development of plasmablastic lymphoma.

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13
Q

Common variable immunodeficiency: Gross appearance of spleen.

A

May be enlarged or unremarkable.

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14
Q

Common variable immunodeficiency: Histology of splenic follicles

A

May be atrophic, hyperplastic, or normal.

May exhibit atypical follicular hyperplasia.

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15
Q

Common variable immunodeficiency: Other possible histologic features (3).

A

Granulomas.

Immunoblasts and Reed-Sternberg-like cells.

Lymphoid hyperplasia in the red pulp.

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16
Q

Common variable immunodeficiency: Viral association.

A

In most cases, EBV is detectable by

  • Immunohistochemical stain for LMP.
  • In-situ hybridization for EBV (EBER).
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17
Q

Common variable immunodeficiency: Lymphocytes.

A

Mixture of T cells and B cells.

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18
Q

Common variable immunodeficiency vs. lymphoma (2).

A

Lymphoma:

  • (Usually) no established history of immunodeficiency.
  • Demonstrated clonality.
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19
Q

Castleman’s disease of the spleen: Viral associations of plasmablastic type.

A

HHV8, with or without HIV.

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20
Q

Autoimmune lymphoproliferative syndrome: Presentation.

A

Generalized lymphadenopathy, splenomegaly, and autoimmune disease in a child under 2 years of age.

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21
Q

Autoimmune lymphoproliferative syndrome: Aetiology.

A

Mutation in Fas, Fas ligand, caspase 8, or caspase 10.

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22
Q

Autoimmune lymphoproliferative syndrome: Manifestation in peripheral blood.

A

Autoimmune cytopenias.

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23
Q

Autoimmune lymphoproliferative syndrome: Complication.

A

Hodgkin’s lymphoma or non-Hodgkin’s lymphoma.

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24
Q

Autoimmune lymphoproliferative syndrome: Histology (3).

A

White pulp: Follicular hyperplasia, expansion of germinal centers.

Red pulp: Expansion by T cells and plasma cells.

PALS: Same as for red pulp.

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25
Q

Autoimmune lymphoproliferative syndrome: Immunohistochemistry (2).

A

Double-negative T lymphocytes, mainly in the red pulp.

No expression of CD25.

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26
Q

Splenic marginal-zone lymphoma: Typical presentation.

A

Massive splenomegaly.

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27
Q

Splenic marginal-zone lymphoma: Manifestations in peripheral blood (2).

A

Autoimmune anaemia.

Thrombocytopenia.

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28
Q

Splenic marginal-zone lymphoma: Additional possible laboratory findings (2).

A

Monoclonal IgM or IgD.

Cryoglobulinemia.

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29
Q

Splenic marginal-zone lymphoma: Affected organs.

A

Splenic hilar lymph nodes, peripheral blood, bone marrow, and liver may all be involved at presentation.

Peripheral lymph nodes are not affected.

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30
Q

Splenic marginal-zone lymphoma: Infectious association.

A

Hepatitis C.

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31
Q

Splenic marginal-zone lymphoma: Gross pathology.

A

Miliary pattern due to expansion of white pulp.

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32
Q

Splenic marginal-zone lymphoma: Histology.

A

White pulp: Expansion of marginal zones, or colonization of germinal centers with attenuation of mantle zones.

Red pulp: Diffuse or nodular infiltrate.

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33
Q

Splenic marginal-zone lymphoma: Cytology (2).

A

Most cells . . .

  • Nucleus: Round to oval.
  • Cytoplasm: Clear, often abundant.

Larger lymphoid cells at the periphery of the nodules.

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34
Q

Splenic marginal-zone lymphoma: Appearance in peripheral blood.

A

Abundant cytoplasm and short polar villi.

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35
Q

Splenic marginal-zone lymphoma: Immunophenotype.

A

Positive: Usual B-cell markers.

Negative: Specific markers.

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36
Q

Splenic marginal-zone lymphoma vs. splenic diffuse red-pulp small B-cell lymphoma

A

SDRPSBCL:

  • Diffuse infiltration of red pulp with obliteration of white pulp.
  • Strongly expresses DBA.44 and often expresses CD11c.
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37
Q

CLL/SLL of the spleen: Gross pathology.

A

Earlier: Miliary pattern due to expansion of white pulp.

Advanced disease: More homogenous appearance due to diffuse disease.

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38
Q

CLL/SLL of the spleen: Histology.

A

White pulp: Diffuse neoplastic infiltrate replaces germinal centers.

Red pulp: Diffuse neoplastic infiltrate.

Proliferation centers are rare in the spleen.

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39
Q

CLL/SLL of the spleen: Types.

A

Pre-germinal-center type:

  • Unmutated IGVH.
  • Often express ZAP-70 and CD38.

Memory-B-cell type: Opposite.

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40
Q

CLL/SLL of the spleen vs. prolymphocytic leukemia: Immunophenotype (4).

A

Prolymphocytic leukemia:

  • Stronger CD20 and sIg.
  • Expression of FMC7.
  • Variable CD5.
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41
Q

Reactive follicular hyperplasia with formation of germinal centers: Histology (3).

A

Follicles consist of a germinal center and well-defined mantle and marginal zones.

Germinal centers are polarized (with dark and light zones) and contain tingible-body macrophages.

Increased plasma cells in the red pulp.

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42
Q

Reactive follicular hyperplasia with formation of germinal centers: Immunohistochemistry of germinal centers (3,1).

A

Positive: CD20, CD10, Bcl-6.

Negative: Bcl-2.

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43
Q

Reactive follicular hyperplasia with formation of germinal centers: Immunohistochemistry of mantle zones (3,2).

A

Positive: CD20, CD5, Bcl-2.

Negative: CD43, cyclin D1.

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44
Q

Relevance of well-developed splenic germinal centers to age.

A

Children and young adults: Normal.

Elderly: May represent lymphoma or autoimmune disease.

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45
Q

Prominent expansion of splenic marginal zone: Cause.

A

Chronic antigenic stimulation.

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46
Q

Prominent expansion of splenic marginal zone vs. marginal-zone lymphoma

A

Marginal-zone lymphoma:

  • Marginal-zone cells infiltrate germinal centers and red pulp.
  • Mantle zone is effaced.
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47
Q

Splenomegaly associated with systemic lupus erythematosus or with rheumatoid arthritis: Histology (3).

A

Follicular hyperplasia.

Plasmacytosis.

Expansion of the red pulp.

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48
Q

Prolymphocytic leukemia: Criterion.

A

At least 55% of circulating lymphocytes are prolymphocytes.

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49
Q

Prolymphocytic leukemia: Presentation (3).

A

Massive splenomegaly.

Cytopenias (due to hypersplenism).

No lymphadenopathy.

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50
Q

Prolymphocytic leukemia: Involved pulp.

A

Diffuse infiltration of red pulp.

White usually also involved.

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51
Q

Prolymphocytic leukemia: Cytology.

A

Prolymphocytes:

  • Medium size.
  • Much cytoplasm.
  • Large nucleolus.
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52
Q

Prolymphocytic leukemia: Immunohistochemistry.

A

B-cell PLL

  • Positive: CD20.
  • Variable: CD5, CD23.

T-cell PLL

  • Positive: CD3, CD4, CD5.
  • May lose T-cell antigens.
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53
Q

Mantle-cell lymphoma: Presentations (2).

A

Usual: Widespread lymphadenopathy, disease in bone marrow.

Splenomegalic: Leukemia, no lymphadenopathy.

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54
Q

Mantle-cell lymphoma: Involved pulp.

A

White pulp.

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55
Q

Mantle-cell lymphoma: Histology.

A

Nodular expansion of white pulp with or without a mantle-zone pattern.

A purely mantle-zone pattern of growth is rare.

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56
Q

Mantle-cell lymphoma: Cytology of blastoid variant.

A

Blastoid cells may resemble lymphoblasts or the large, pleomorphic cells or DLBCL.

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57
Q

Mantle-cell lymphoma: Immunophenotype (4,1).

A

Positive: CD20, CD5, cyclin D1, SOX11.

Negative: CD23.

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58
Q

Mantle-cell lymphoma: Function of cyclin D1.

A

Regulates progression of G1 phase to S phase.

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59
Q

Follicular lymphoma: Frequency of splenic disease.

A

About 50%.

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60
Q

Follicular lymphoma: Involvement of pulp.

A

Involves white pulp mainly.

The red pulp may contain small aggregates of lymphoma cells.

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61
Q

Follicular lymphoma: Frequency of lack of expression of Bcl-2.

A

About 20%.

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62
Q

Diffuse large B-cell lymphoma: Gross pathology (2).

A

Large nodules of tumor, often with necrosis.

Involvement of hilar and retroperitoneal lymph nodes.

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63
Q

Diffuse large B-cell lymphoma: Involved pulp.

A

Involves both white and red pulp, effacing the splenic architecture.

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64
Q

Hodgkin’s lymphoma: Frequency of disease in the spleen (2).

A

Primary splenic Hodgkin’s lymphoma is exceedingly rare.

NLP-HD rarely involves the spleen.

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65
Q

Gaucher’s disease:

A. Defective enzyme.
B. Storage product.

A

A. Glucocerebrosidase (beta-glucosidase).

B. Glucocerebroside.

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66
Q

Gaucher’s disease: Clinical forms.

A

Infantile form: Hepatosplenomegaly, mental deterioration, death in infancy of early childhood.

Adult form.

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67
Q

Gaucher’s disease, adult form:

A. Epidemiology.
B. Age of onset.
C. Presentation.

A

A. Most common in Ashkenazi Jews.

B. Late childhood or adulthood.

C. No mental retardation; pancytopenia may occur.

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68
Q

Gaucher’s disease: Affected pulp.

A

Red pulp.

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69
Q

Gaucher’s disease, adult form: Affected organs (4).

A

Liver.

Spleen.

Adrenal glands.

Bones.

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70
Q

Gaucher’s disease: Cytology.

A

Brown, “wrinkled-silk” cytoplasm.

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71
Q

Gaucher’s disease: Special stains (4).

A

Positive: PAS with and without diastase, iron stain.

Colorless on Wright’s stain.

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72
Q

Gaucher’s disease: Histologic differential diagnosis (2).

A

Niemann-Pick disease: Foamy or bubbly cytoplasm.

Ceroid histiocytes: Faintly yellow-brown on H and E but blue-green on Wright-Giemsa stain.

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73
Q

Gaucher’s disease: Electron microscopy.

A

Lysosomes contain twisted helical tubules.

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74
Q

Niemann-Pick disease:

A. Defective enzyme.
B. Storage product.

A

A. Sphingomyelinase.

B. Sphingomyelin.

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75
Q

Niemann-Pick disease: Affected organs (5).

A

Spleen.

Liver.

Lungs.

Brain.

Bone marrow.

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76
Q

Niemann-Pick disease: Cytology.

A

Yellow-green, foamy or bubbly cytoplasm.

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77
Q

Niemann-Pick disease: Special stains (2).

A

Positive: AFB stain.

Blue-green on Wright-Giemsa stain.

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78
Q

Niemann-Pick disease: Electron microscopy.

A

Variably sized residual bodies.

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79
Q

Mucopolysaccharidoses:

A. Appearance on routine stain.
B. Special stain.

A

A. Clear cytoplasm.

B. Weakly positive: PAS without diastase.

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80
Q

Mucopolysaccharidoses: Electron microscopy.

A

Membrane-bound vacuoles with lamellar inclusions.

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81
Q

Glycogen-storage diseases:

A. Appearance on routine stain.
B. Special stain.

A

A. Clear cytoplasm.

B. Positive: PAS without diastase.

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82
Q

Glycogen-storage diseases: Electron microscopy.

A

Glycogen granules.

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83
Q

Extramedullary hematopoiesis: Types.

A

Non-neoplastic.

Neoplastic: The extramedullary hematopoiesis is itself malignant.

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84
Q

Extramedullary hematopoiesis, non-neoplastic: Causes (3).

A

Severe anemia.

Myelophthisis

Normal fetal state or prematurity.

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85
Q

Extramedullary hematopoiesis, non-neoplastic: Presentation.

A

Usually asymptomatic; incidental finding.

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86
Q

Extramedullary hematopoiesis, neoplastic type: Presentation.

A

Pain due to splenomegaly or splenic infarcts.

Cytopenias.

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87
Q

Extramedullary hematopoiesis, non-neoplastic: Causes (2).

A

Myeloproliferative neoplasms, esp. primary myelofibrosis.

MDS/MPNs.

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88
Q

Extramedullary hematopoiesis, non-neoplastic: Differential diagnosis.

A

Non-neoplastic EMH.

Extramedullary acute leukemia.

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89
Q

Extramedullary hematopoiesis: Affected pulp.

A

Red pulp.

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90
Q

Hereditary spherocytosis: Histology (4).

A

Cords: Distended; increased macrophages.

Sinuses: Empty; hypertrophic lining cells.

Little erythrophagocytosis.

Minimal hemosiderin.

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91
Q

Fibrocongestive splenomegaly: Association.

A

Liver disease.

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92
Q

Fibrocongestive splenomegaly:

A. Histology.
B. Differential diagnosis.

A

A. Fibrosis and congestion.

B. Capillary hemangioma and splenic hamartoma are usually better circumscribed.

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93
Q

Sickle-cell disease: Causes of sepsis.

A

Haemophilus influenzae.

Streptococcus pneumoniae.

Neisseria meningitidis.

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94
Q

Gross appearance of spleen in ___.

A. HbSS disease.
B. Hemoglobin C disease.

A

A. Shrunken and fibrotic due to total infarction.

B. Possibly enlarged; infarction may be focal but is never total.

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95
Q

Sickle-cell disease: First part of the spleen to be affected.

A

The follicles.

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96
Q

Gamna-Gandy body.

A

Old organized microinfarct that is encrusted with iron.

Typical but not specific for sickle-cell disease.

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97
Q

Autoimmune hemolytic anemia: Histology (4).

A

White pulp: Follicular hyperplasia.

Red pulp:

  • Increased plasma cells.
  • Erythrophagocytosis.
  • Congested cords; empty-appearing sinuses.
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98
Q

Evans’ syndrome.

A

ITP + hemolytic anemia.

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99
Q

Immune thrombocytopenic purpura: Clinical forms.

A

Acute: More common in children.

Chronic: More common in middle-aged.

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100
Q

Immune thrombocytopenic purpura: Histology of spleen (4).

A

White pulp: Follicular hyperplasia.

Red pulp:

  • “Dirty”-appearing cordal macrophages due to ingested platelets.
  • Foamy macrophages.
  • Increased neutrophils.
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101
Q

Immune thrombocytopenic purpura: Histology of bone marrow.

A

Megakaryocytes may be increased.

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102
Q

Immune thrombocytopenic purpura: Role of spleen (2).

A

Destruction of platelets.

Production of anti-platelet antibodies.

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103
Q

Immune thrombocytopenic purpura: Indication of splenectomy.

A

Failure to respond to steroids.

104
Q

Splenic diffuse red-pulp small B-cell lymphoma: Involved pulp.

A

Red pulp.

White pulp is atrophic.

105
Q

Splenic diffuse red-pulp small B-cell lymphoma: Cytology (2).

A

Small to medium-sized lymphoid cells.

Nucleus: Round; occasional small distinct nucleoli.

106
Q

Splenic diffuse red-pulp small B-cell lymphoma: Other involved organs (3).

A

Bone marrow: Intrasinusoidal infiltrate.

Peripheral blood: Villous lymphocytes.

Skin: Dermal infiltrate surrounding adnexa and blood vessels; epidermotropism.

107
Q

Splenic diffuse red-pulp small B-cell lymphoma vs. splenic marginal-zone lymphoma.

A

Splenic marginal-zone lymphoma involves both white pulp and red pulp.

108
Q

Splenic diffuse red-pulp small B-cell lymphoma vs. hairy-cell-leukemia variant.

A

HCL-v: At least some lymphoid cells have large nucleoli.

109
Q

Splenic diffuse red-pulp small B-cell lymphoma: Immunohistochemistry (1,2,2).

A

Positive: DBA.44.

Variable: CD103, CD11c.

Negative: Annexin A-1, CD25.

110
Q

Hairy-cell leukemia: Clinical presentation (4).

A

Splenomegaly, anemia, infections.

No lymphadenopathy.

111
Q

Hairy-cell leukemia: Most specific marker.

A

CD103.

112
Q

Hairy-cell leukemia: Involved pulp.

A

Red pulp.

White pulp may be obliterated.

113
Q

Hairy-cell leukemia: Histology.

A

Hairy cells in cord and sinuses.

Hairy cells form periphery of blood lakes.

114
Q

Hairy-cell-leukemia: Cytology.

A

Nucleus: Inconspicuous nucleolus.

Cytoplasm: Villi.

115
Q

Hairy-cell leukemia: Morphologic variants.

A

Larger cells.

Blastoid cells.

116
Q

Hairy-cell leukemia: Special stain.

A

Reticulin stain reveals fibers surrounding the hairy cells.

117
Q

Hairy-cell leukemia: Immunophenotype (6).

A

Positive: CD103, CD11c, CD25, TRAP, DBA.44, annexin A1, FMC7.

118
Q

Hairy-cell leukemia: Mutation.

A

V600E in BRAF.

119
Q

Hairy-cell-leukemia variant: Sites of involvement.

A

Spleen.

Peripheral blood.

Bone marrow: More easily aspirated than in classic HCL.

Liver and lymph nodes are usually spared.

120
Q

Hairy-cell-leukemia variant: Involved pulp.

A

Red pulp.

White pulp is atrophic.

121
Q

Hairy-cell-leukemia variant: Histology.

A

Blood lakes may be visible.

122
Q

Hairy-cell-leukemia variant: Cytology.

A

Nucleus: Round or bilobate; large nucleolus.

Cytoplasm: Basophilic; many villi.

May resemble prolymphocytic leukemia or classic HCL.

123
Q

Hepatosplenic T-cell lymphoma: Association.

A

Prolonged immunosuppression.

Lymphoma may first appear in a transplanted organ.

124
Q

Hepatosplenic T-cell lymphoma: Presentation (3).

A

Hepatosplenomegaly.

Cytopenias.

“B” symptoms.

125
Q

Hepatosplenic T-cell lymphoma: Involved pulp.

A

Red pulp.

White pulp is obliterated.

126
Q

Hepatosplenic T-cell lymphoma: Histology.

A

Tumor cells distend sinuses and may form sheets.

127
Q

Hepatosplenic T-cell lymphoma: Cytology.

A

Nucleus: Oval or folded; inconspicuous nucleolus.

Cytoplasm: Pale.

128
Q

Hairy-cell-leukemia variant: Immunophenotype (2,1,3).

A

Positive: DBA.44, CD11c.

Variable: CD103.

Negative: CD25, TRAP, annexin A1.

129
Q

Hepatosplenic T-cell lymphoma: Pattern of disease in bone marrow.

A

Intravascular.

Clusters of tumor cells.

130
Q

Hepatosplenic T-cell lymphoma: Expressed markers (3).

A

CD3, CD56, TIA-1.

131
Q

Hepatosplenic T-cell lymphoma: Unexpressed markers (6).

A

CD4, CD8.

CD5.

CD57.

Granzyme B, perforin.

132
Q

Hepatosplenic T-cell lymphoma: Flow cytometry.

A

Most cases have the gamma-delta TCR.

A few have the alpha-beta TCR.

133
Q

Large-granular-lymphocytic leukemia: Presentation (3).

A

Neutropenia with or without anemia.

Moderate splenomegaly.

Variable lymphocytosis.

134
Q

Large-granular-lymphocytic leukemia: Immunophenotypes.

A

T-LGL expresses CD3, CD8, alpha-beta TCR, granzyme B.

NK-LGL expresses CD16, CD56, cCD3.

135
Q

Hepatosplenic T-cell lymphoma: Prognosis.

A

Median survival is less than 2 years.

136
Q

Hepatosplenic T-cell lymphoma: Mutation.

A

i(7q).

137
Q

Systemic mastocytosis: Involved pulp.

A

Red pulp.

138
Q

Systemic mastocytosis:

A. Composition of nodules.
B. Location of nodules.

A

A. Mast cells, eosinophils, fibrosis.

B. Perivascular, peritrabecular, perifollicular.

139
Q

Systemic mastocytosis: Possible morphologies of mast cells (4).

A

Spindled.

May resemble

  • Monocytes.
  • Monocytoid B cells.
  • Hairy cells.
140
Q

Systemic mastocytosis: Immunophenotypic abnormality.

A

Expression of CD25 and (often) CD2.

141
Q

Systemic mastocytosis: Laboratory finding.

A

Elevated serum tryptase.

142
Q

Langerhans’-cell histiocytosis: Involved pulp.

A

Red pulp.

143
Q

Langerhans’-cell histiocytosis: Patterns of infiltrate (2).

A

Diffuse.

Multinodular (granulomatoid).

Discrete tumor masses are rare.

144
Q

Langerhans’-cell histiocytosis: Nucleus of tumor cell.

A

Pale and bean-shaped.

145
Q

Langerhans’-cell histiocytosis: Accompanying cells.

A

Eosinophils and plasma cells, although much less numerous than in “eosinophilic granuloma”.

146
Q

Systemic mastocytosis: Mutation.

A

D816V in KIT.

147
Q

Histiocytic sarcoma: Presentation.

A

Serious illness; cytopenias.

148
Q

Histiocytic sarcoma: Involved pulp.

A

Red pulp.

Bone marrow is usually also involved.

149
Q

Histiocytic sarcoma: Cytology.

A

Highly atypical cells with large nucleoli and ,any mitotic figures.

150
Q

Histiocytic sarcoma: Additional histologic feature.

A

Erythrophagocytosis by macrophages (but not by tumor cells).

151
Q

Histiocytic sarcoma: Immunohistochemistry (2,1,1).

A

Positive: CD68, CD163.

Variable: S100.

Negative: CD1a.

152
Q

Splenic hemangioma: Possible complications (2).

A

Cytopenias due to hypersplenism.

Consumptive coagulopathy (DIC).

153
Q

Splenic hemangioma: Most common type.

A

Cavernous.

154
Q

Splenic hemangioma: Incompatible histologic features (2).

A

Endothelial tufting.

Endothelial mitotic figures (except in children).

155
Q

Splenic hemangioma: Immunohistochemistry (1,2).

A

Positive: Vascular markers.

Negative: Hematopoietic markers, Ki-67.

156
Q

Splenic angiomatosis.

A

Diffuse replacement of spleen by hemangioma.

157
Q

Peliosis of the spleen: Association.

A

Peliosis hepatis.

158
Q

Peliosis of the spleen:

A. Location.
B. Possible complication.

A

A. Diffuse or multifocal in the spleen.

B. Splenic rupture.

159
Q

Peliosis of the spleen: Histology.

A

Dilated sinuses lined by flattened cells and surrounded by splenic parenchyma.

No fibrosis.

160
Q

Peliosis of the spleen: Immunohistochemistry (2).

A

Positive: CD68, CD8.

161
Q

Myoid angioendothelioma: Histology.

A

Consists of

  • Vascular spaces.
  • Stromal cells with myoid features.
162
Q

Vascular marker that is usually not expressed by lymphatic vessels.

A

CD34.

163
Q

Littoral-cell angioma: Presentation.

A

May cause splenomegaly and hypersplenism.

164
Q

Littoral-cell angioma: Gross pathology.

A

May be multiple (in contrast to hemangioma); rarely replaces the whole spleen.

165
Q

Littoral-cell angioma: Architecture (2).

A

Variable-shaped vascular spaces lined by littoral cells.

Papillae of littoral cells may project into vascular spaces.

166
Q

Littoral-cell angioma: Cytology (2).

A

Bland, cuboidal or hobnail-shaped cells with few mitotic figures.

Nuclei: Large and vesicular.

167
Q

Littoral-cell angioma: Incompatible histologic features (2).

A

Solid growth.

Necrosis.

168
Q

Littoral-cell angioma: Special stains (2).

A

Reticulin: Annular fibers around vascular spaces.

PAS: Cytoplasmic globules.

169
Q

Littoral-cell angioma: Expressed markers (6).

A

CD31, CD68, CD68R, CD21, CD163.

S100 sometimes.

170
Q

Littoral-cell angioma vs. normal littoral cells: Immunophenotype.

A

Normal littoral cells express CD8.

171
Q

Littoral-cell angioma: Unexpressed markers (2).

A

CD8, CD34.

172
Q

Splenic angiosarcoma: Associations.

A

Not associated with thorium dioxide or with vinyl chloride.

173
Q

Splenic angiosarcoma: Gross pathology (2).

A

Splenomegaly, often marked.

May be multiple.

174
Q

Splenic angiosarcoma: Architecture (3).

A

Irregular anastomosing vascular channels.

May show solid growth.

Papillae may project into vascular spaces.

175
Q

Splenic angiosarcoma: Cytology

A

Variable shape of cells.

Mild to severe atypia.

Variable mitotic activity.

176
Q

Splenic angiosarcoma: Possible additional histologic features (2).

A

Extramedullary hematopoiesis.

Erythrophagocytosis.

177
Q

Splenic angiosarcoma: Most consistently expressed vascular markers.

A

CD31.

Ulex lectin.

178
Q

Splenic angiosarcoma: Electron microscopy.

A

Weibel-Palade bodies.

179
Q

Splenic angiosarcoma vs. hemangioendothelioma (4).

A

Hemangioendothelioma:

  • No necrosis.
  • No solid growth.
  • Less atypia.
  • Fewer mitotic figures.
180
Q

Splenic angiosarcoma vs. hemangiopericytoma (4).

A

Hemangiopericytoma:

  • No necrosis.
  • Little or no atypia.
  • Fewer mitotic figures.
  • Spindle cells surround rather than form vascular spaces.
181
Q

Splenic angiosarcoma vs. bacillary angiomatosis (4).

A

Bacillary angiomatosis:

  • No anastomosing vascular channels.
  • Little or no atypia.
  • Few mitotic figures.
  • Basophilic clumps of bacteria.
182
Q

Epidermoid cyst of the spleen: Presentation.

A

Asymptomatic unless infected.

183
Q

Littoral-cell angioma vs. hemangioendothelioma (2).

A

Hemangioendothelioma:

  • Hyperchromatic nuclei.
  • Usually no expression of CD68.
184
Q

Epidermoid cyst of the spleen: Gross pathology (2).

A

Lining: Shiny, trabeculated.

Contents: Turbid yellow fluid.

185
Q

Epidermoid cyst of the spleen: Histology.

A

Thin wall lined by squamous, transitional, or columnar epithelium.

186
Q

Epidermoid cyst of the spleen: Putative histologic origin.

A

Mesothelium.

187
Q

Pseudocyst of the spleen: Presentation.

A

Asymptomatic unless infected.

188
Q

Pseudocyst of the spleen: Putative aetiology.

A

Degeneration of traumatic splenic hematoma or of splenic infarct.

189
Q

Pseudocyst of the spleen: Gross pathology (2).

A

Lining: Smooth (not trabeculated).

Contents: Dark red-brown fluid.

190
Q

Pseudocyst of the spleen: Histology (2).

A

Fibrous wall without epithelial lining.

Calcifications in wall.

191
Q

Echinococcal cyst of the spleen: Risk factors (2).

A

Exposure to livestock or deer.

Exposure to feces of canids.

192
Q

Echinococcal cyst of the spleen: Gross pathology (3).

A

Often multilocular.

Granular lining.

Granules in the contents.

193
Q

Splenic hamartoma: Gross pathology.

A

Usually solitary, well-circumscribed, red, bulging nodule.

194
Q

Splenic hamartoma: Histology

A

Consists of elements of red pulp: Cord-like and sinus-like structure.

Compressed red pulp at the periphery, but no capsule.

May contain foci of infarction and fibrosis.

195
Q

Splenic hamartoma, cordal variant.

A

Consists mainly of cordal macrophages.

May resemble inflammatory pseudotumor.

196
Q

Splenic hamartoma: Immunohistochemistry of normal splenic endothelium (2,1).

A

Positive: CD8, CD68.

Negative: CD34.

197
Q

Inflammatory pseudotumor of the spleen: Gross pathology.

A

Usually solitary, well-circumscribed, pale, bulging nodule, often with central necrosis.

198
Q

Inflammatory pseudotumor of the spleen: Variants.

A

“Truly inflammatory” type: Older patients.

Hepatosplenic-IPT-like tumor of follicular dendritic cells: Mostly in females.

199
Q

Inflammatory pseudotumor of the spleen: Cellular components.

A

Bland spindle cells.

Lymphocytes, plasma cells, macrophages, neutrophils.

Few or no eosinophils.

200
Q

Inflammatory pseudotumor of the spleen: Association.

A

Hepatosplenic-IPT-like tumor of follicular dendritic cells consistently harbors EBV-DNA.

201
Q

Inflammatory pseudotumor of the spleen: General immunohistochemistry (2,1).

A

Positive: MSA, SMA.

Variable: Desmin.

202
Q

Inflammatory pseudotumor of the spleen: Special immunohistochemistry.

A

“Truly inflammatory variant”: Expression of CD68 but not of FDC markers.

Hepatosplenic-IPT-like tumor of follicular dendritic cells: Expression of CD21, CD23, CD35, EBV-LMP.

203
Q

Inflammatory pseudotumor of the spleen: Additional immunohistochemistry.

A

An increased ratio of IgG4 to IgG should prompt a search for evidence (e.g. chronic pancreatitis) of IgG4-related disease.

204
Q

Inflammatory pseudotumor of the spleen: Differential diagnosis in patients with AIDS (2).

A

Mycobacterial pseudotumor.

Bacillary angiomatosis.

205
Q

Inflammatory pseudotumor of the spleen vs. inflammatory myofibroblastic tumor.

A

Inflammatory myofibroblastic tumor:

  • Clonal myofibroblasts.
  • Expresses ALK.
206
Q

Congestive splenomegaly: Causes (2).

A

Cirrhosis of the liver.

Thrombosis of the splenic vein (PNH, PV).

207
Q

Congestive splenomegaly: Gross pathology (2).

A

Expanded red pulp; inconspicuous white pulp.

Spleen usually weighs less than 1 kg.

208
Q

Congestive splenomegaly: Evolution of histology.

A

Early: Cellular red pulp.

Later:

  • Hypocellular red pulp with reticulin fibrosis.
  • Dilated sinuses due to fibrotic retraction.
  • Gamna-Gandy bodies may occur.
209
Q

Fibrocongestive splenomegaly:

A. Synonym.
B. Immunohistochemistry.

A

A. Chronic passive congestion of the spleen.

B. Increased expression of SMA due to proliferation of splenic myoid cells.

210
Q

Congestive splenomegaly vs. splenic hamartoma.

A

Splenic hamartoma is well circumscribed.

211
Q

Splenic hamartoma vs. hemangioma.

A

Hemangioma

  • Contains organized vascular spaces.
  • Expresses CD34 but not CD8 or CD68.
212
Q

Vasculitis in the spleen: Gross pathology (2).

A

Infarcts.

Splenic rupture has been reported.

213
Q

Polyarteritis nodosa: Type of affected blood vessel.

A

Small artery.

214
Q

Polyarteritis nodosa: Histology (2).

A

Fibrinoid necrosis.

Neutrophils and eosinophils in the walls of the vessels.

215
Q

Churg-Strauss syndrome: Synonym.

A

Hypersensitivity angiitis.

216
Q

Churg-Strauss syndrome: Histology in the spleen (3).

A

Arterioles: Leukocytoclastic vasculitis.

Small vessels: Fibrinoid necrosis.

Eosinophils.

217
Q

Systemic lupus erythematosus: Histology in the spleen (4).

A

Arterioles:

  • Leukocytoclastic vasculitis.
  • Perivascular fibrosis (“onion-skin” appearance).

Small vessels: Fibrinoid necrosis.

Plasmacytosis in red pulp.

218
Q

Rheumatoid arthritis: Histology in the spleen (3).

A

Arterioles: Leukocytoclastic vasculitis.

Small vessels: Fibrinoid necrosis.

Follicular hyperplasia in the white pulp.

219
Q

TTP: Histology in the spleen (4).

A

Arterioles: Perivascular fibrosis sometimes.

Small vessels:

  • Thrombi consisting of platelets and fibrin.
  • No inflammation.

Subendothelial PAS-positive hyaline deposits.

220
Q

Thromboemboli: Types that cause true vasculitis.

A

Septic emboli.

Emboli of endocarditis.

221
Q

Infectious mononucleosis: Gross pathology.

A

Red pulp: Congestion.

White pulp: Hyperplasia.

Spontaneous rupture can occur.

222
Q

Infectious mononucleosis: Histology (4).

A

Red pulp: Expansion by lymphocytes, immunoblasts, plasma cells.

White pulp: Follicular hyperplasia.

Periarteriolar lymphoid sheaths: Lymphoid cells including immunoblasts.

Lymphoid cells may infiltrate trabeculae and capsule.

223
Q

Infectious mononucleosis: Immunohistochemistry of immunoblasts (2,1).

A

Positive: CD20, EBV.

Often positive: CD30.

224
Q

Infectious mononucleosis: T lymphocytes.

A

Mainly cytotoxic, CD8+.

225
Q

Infectious mononucleosis vs. lymphoma: Gross pathology.

A

Lymphomas (both Hodgkin’s and non-Hodgkin’s) tend to form discrete masses in the spleen.

226
Q

Cytomegalovirus infection of the spleen: Complication.

A

Virus-associated hematophagocytic syndrome: Fever, chills, malaise, and pancytopenia.

227
Q

Cytomegalovirus infection of the spleen: Gross pathology.

A

Red pulp: Congestion.

White pulp: Usually inconspicuous.

228
Q

Mycobacterium avium-intracellulare infection of the spleen: Laboratory finding.

A

Anemia.

229
Q

Mycobacterium avium-intracellulare infection of the spleen: Involved pulp.

A

Red pulp: Firmly expanded.

230
Q

Mycobacterium avium-intracellulare infection of the spleen: Histology (2).

A

Histiocytes appear pale gray on routine stain.

There may be erythrophagocytosis.

231
Q

Mycobacterium avium-intracellulare infection of the spleen: Special stains.

A

Tissue: AFB, PAS.

Cytology: Wright, Papanicolaou.

232
Q

Malaria in the spleen: Leading cause of splenomegaly.

A

Plasmodium vivax.

233
Q

Malaria in the spleen, acute phase: Gross pathology.

A

Red-brown parenchyma due to congestion and malarial pigment.

234
Q

Malaria in the spleen, acute phase: Histology

A

Venous sinuses engorged with parasitized red cells.

Sinus macrophages contain hemosiderin, red cells, malarial pigment.

Increase small lymphocytes in the red pulp.

235
Q

Malaria in the spleen, acute phase: Cells that contain parasites.

A

Erythrocytes.

Sinus-lining cells sometimes.

236
Q

Malaria in the spleen, chronic phase: Gross pathology.

A

Gray parenchyma with fibrosis and scarring.

237
Q

Malaria in the spleen, chronic phase: Histology (2).

A

Fibrosis and scarring.

Pigment-laden macrophages in the PALS.

238
Q

Hyperactive malarial splenomegaly: Association.

A

Chronic malarial infection in endemic areas.

239
Q

Hyperactive malarial splenomegaly: Histology (2).

A

Hyperplasia of sinusoids and of reticuloendothelial system.

Intense splenic sequestration with erythrophagocytosis.

240
Q

Malaria in the spleen, chronic phase: Another possible manifestation.

A

Splenic pseudocyst.

241
Q

Properties of malarial pigment (3).

A

Refractile.

Birefringent.

Negative on iron stain.

242
Q

Abscess vs. infarct:

A. Consistency.
B. Location of greatest inflammation.

A

A. Abscess is soft or liquid; infarct is firm.

B. In the center of an abscess; in the periphery of an infarct.

243
Q

Miliary tuberculosis of the spleen: Gross pathology.

A

Granulomas are often less well formed and lack visible necrosis.

244
Q

Lipogranulomatosis.

A

Presence of lipid matter derived endogenous sources, e.g. tumors, hematomas, cholesterol deposits.

245
Q

Lipogranulomatosis:

A. Gross pathology.
B. Histology.

A

A. Not seen grossly.

B. Lipid vacuoles surrounded by macrophages; located near arterioles in the white pulp.

246
Q

Splenic ___: Affected pulp.

A. sarcoidosis
B. miliary tuberculosis
C. histoplasmosis
D. coccidioidomycosis

A

A. White pulp.

B. Both.

C. Red pulp more than white pulp.

D. Both.

247
Q

Splenic histoplasmosis, acute phase: Histology (3).

A

May lack discrete granulomas.

Histiocytes, plasma cells, lymphocytes.

Usually no neutrophils.

248
Q

Splenic histoplasmosis, inactive phase: Histology (2).

A

Calcification and fibrosis surrounded by a few lymphocytes.

Usually no histiocytes.

249
Q

Origin of __ amyloid.

A. AL.
B. AA.

A

A. Immunoglobulin light chains, especially lambda.

B. SAA protein.

250
Q

Causes of AA-type amyloidosis (3).

A

Tuberculosis.

Rheumatoid arthritis.

Other chronic inflammatory diseases.

251
Q

Splenic amyloidosis: Gross pathology.

A

Incidental: Grossly inapparent.

Sago spleen: Nodular.

Lardaceous spleen: Diffuse.

252
Q

Splenic amyloidosis: Location of deposits.

A

Incidental: Around small vessels.

Sago spleen: White pulp.

Lardaceous spleen: Red pulp near sinuses and around small vessels.

253
Q

Familial Mediterranean fever: Gene.

A

MEFV.

254
Q

Splenic hyalinosis:

A. Age group.
B. Histology.
C. Cause.

A

A. Any age after early childhood.

B. Hyaline thickening of small arteries and arterioles.

C. Deposition of plasma proteins.

255
Q

Amyloidosis vs. splenic hyalinosis.

A

Splenic hyalinosis: No reaction with Congo red or thioflavin T stain.

256
Q

Splenomegaly associated with systemic lupus erythematosus or with rheumatoid arthritis: Immunophenotype.

A

T lymphocytes that cause expansion of the red pulp lack CD3 and CD8 and express CD57.

AP reviewer (17 decks)

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