PATHO - WBC and Lymph Nodes 2 (NK and T, Plasma Cells)

5-10%

Percentage of NHLs made up by peripheral T-cell tumors?

Asia

Geographic region where NK tumors are more common?

Activated CD8 T cell

Cell of origin of ALK-positive anaplastic large cell lymphoma?

ALK+, CD30+

Key surface markers of ALK-positive lymphoma?

Horseshoe-shaped

Shape of hallmark nuclei in ALK lymphoma?

Voluminous

Cytoplasm description of hallmark cells?

ALK protein

Gene product indicating ALK rearrangement?

Children or young adults

Age group commonly affected by ALK-positive variant?

Older adults

Age group for ALK-negative variant?

Good

Prognosis of ALK-positive variant?

Poor

Prognosis of ALK-negative variant?

75-80%

Chemotherapy cure rate in ALK-positive lymphoma?

HTLV-1

Virus associated with Adult T-cell Leukemia/Lymphoma?

Skin lesions

Common skin manifestation of Adult T-cell Leukemia/Lymphoma?

Lymphocytosis

Common hematologic feature?

Hepatosplenomegaly

Two organomegaly findings in Adult T-cell Leukemia/Lymphoma?

Flower cell

Characteristic abnormal cell seen in blood smear?

Multilobulated

Nuclei appearance of flower cells?

Fatal

Prognosis of Adult T-cell Leukemia/Lymphoma?

Months to 1 year

Average survival of Adult T-cell Leukemia/Lymphoma?

Skin

Main organ system involved in Mycosis Fungoides?

Three

Number of stages in Mycosis Fungoides?

Premycotic

First phase of Mycosis Fungoides?

Plaque phase

Second phase of Mycosis Fungoides?

Tumor phase

Final phase of Mycosis Fungoides?

CD4 T cells

Cell type infiltrating skin lesions in Mycosis Fungoides?

Sezary syndrome

Variant of Mycosis Fungoides with erythroderma?

Exfoliative erythroderma

Primary feature of Sezary syndrome?

Lymph nodes and marrow

Typical spread of advanced Mycosis Fungoides?

10 years

Median survival of Mycosis Fungoides?

Aggressive T-cell lymphoma

Terminal event in Mycosis Fungoides progression?

Nasopharynx

Predominant site of extranodal NK/T-cell lymphoma?

Vascular destruction

Histologic hallmark feature of extranodal NK/T-cell lymphoma?

EBV

Frequent virus association of NK/T-cell lymphoma?

NK markers

NK/T-cell lymphoma commonly expresses which cell marker type?

Negative

CD3 expression status in NK/T-cell lymphoma?

Necrosis

Major tissue finding due to vascular invasion in extranodal NK/T-cell lymphoma?

Good

Typical response to radiation therapy extranodal NK/T-cell lymphoma?

Poor

Typical response to chemotherapy extranodal NK/T-cell lymphoma?

Poor

Prognosis in advanced NK/T-cell lymphoma?

B cell

Type of cell neoplasm represented by plasma cell neoplasms?

Monoclonal Ig secretion

Key characteristic of plasma cell neoplasms?

Bone marrow

Primary site of origin for plasma cell neoplasms?

M protein

Common marker protein for monoclonal immunoglobulins?

Bence Jones proteins

Light chains excreted in urine in plasma cell neoplasms?

Paraproteinemia

Term for serum monoclonal immunoglobulin increase?

Multiple myeloma

Most important and common plasma cell neoplasm?

65-70 years

Typical age range for multiple myeloma?

Interleukin-6

Cytokine critical for myeloma cell survival?

Poor

High IL-6 levels indicate what prognosis in multiple myeloma?

Lytic lesions

Key bone pathology in multiple myeloma?

Vertebral column

Most commonly involved bone in multiple myeloma?

Punched-out lesions

Radiologic appearance of skull lesions in multiple myeloma?

Plasmacytosis

Bone marrow finding in multiple myeloma?

Mott cell

Plasma cell variant with grape-like inclusions in multiple myeloma?

Russell body

Inclusion found within cytoplasm of plasma cells in multiple myeloma?

Dutcher body

Nuclear inclusion in plasma cells in multiple myeloma?

Flame cell

Cell type with fiery red cytoplasm in multiple myeloma?

Elevated M protein

Cause of Rouleaux formation in multiple myeloma?

Stacked coins

Appearance of red cells in Rouleaux formation?

Lupus erythematosus

Non-specific disease that may also show Rouleaux?

Myeloma kidney

Renal complication caused by Bence Jones proteins?

Light chains

Renal amyloidosis in myeloma results from deposition of what?

Congo red

Diagnostic stain for amyloid deposits?

Apple-green

Birefringence color of amyloid under polarized light?

CD138

Plasma cell marker expressed in multiple myeloma?

CD19, CD20

Common pan-B cell markers absent in multiple myeloma?

IgG

Predominant immunoglobulin produced in multiple myeloma?

IgA

Second most common immunoglobulin type produced in multiple myeloma?

Hyperviscosity syndrome

Complication due to thickened blood from excessive Ig?

M proteins

Non-secretory myeloma produces no what?

Renal failure

Major cause of death in multiple myeloma?

4-7 years

Typical median survival for multiple myeloma?

6-12 months

Prognosis of untreated multiple bony lesion myeloma?

65-70 years

Age range commonly affected by lymphoplasmacytic lymphoma?

Lymphocytic and plasma cell

Two major components of lymphoplasmacytic lymphoma?

IgM

Immunoglobulin most commonly secreted?

Waldenstrom macroglobulinemia

Name of syndrome caused by high serum IgM?

Absent

Bone destruction presence in lymphoplasmacytic lymphoma?

Russell body

Cytoplasmic immunoglobulin inclusion?

Dutcher body

Nuclear immunoglobulin inclusion?

CD20

B-cell marker expressed by lymphoid component?

IgM

Predominant secreted immunoglobulin in plasma component?

Waldenstrom macroglobulinemia

Syndrome caused by hyperviscosity due to IgM?

Visual impairement

Common visual symptom in Waldenström macroglobulinemia?

Dizziness

Neurologic manifestation due to sluggish blood flow?

Platelet interference

Type of bleeding disorder from macroglobulins?

Cryoglobulinemia

Cold-induced precipitation of macroglobulins?

Raynaud phenomenon

Cold sensitivity symptom due to cryoglobulinemia?

Lytic lesions

Key bone manifestation of multiple myeloma?

Pathologic fractures

Common skeletal complication of multiple myeloma?

Amyloidosis

Renal complication due to immunoglobulin deposition?

Primary AL

Type of amyloidosis seen in multiple myeloma?

Bence Jones proteins

Light chain component responsible for amyloid formation?

Impaired humoral immunity

Primary immunologic defect in multiple myeloma?

IgM

Predominant immunoglobulin in lymphoplasmacytic lymphoma?

Waldenstrom macroglobulinemia

Syndrome characterized by high IgM viscosity?

HTLV-1

Virus associated with Adult T-cell Leukemia/Lymphoma?

EBV

Virus associated with NK/T-cell lymphoma?

IL-6

Cytokine linked to bone destruction in multiple myeloma?