Page 440 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

424 PART 6 ■ Neoplastic Disorders

Immunohistological Features
TABLE 22.4
of Selected T-Cell Neoplasms

Surface Membrane Markers

Type of Neoplasm CD3 CD4 CD5 CD8

T-PLL + ± − −/+

T-LGL + − − +

Mycosis fungoides/ + + + −

Sézary syndrome

T-PLL, T-cell prolymphocytic leukemia/lymphoma; T-LGL, T-cell large gran-
ular lymphocytic leukemia; +, >90%; ±, >50%; −/+, <50%; −, <10%.

Source: Handin RI, et al. (eds.). Blood: Principles and Practice of

Hematology, 2nd ed, Philadelphia, PA: Lippincott Williams & Wilkins, 2003.

FIGURE 22.5 -cell l rge gr nul r ly phocytic leuke i . Bone
rrow core biopsy reve ls ly phoi ggreg te with p le ger i-

cell orphology (Fig. 22.5), i unophenotyping, n n l center n interstiti l ly phoi inf ltr tion in the j cent re .

olecul r genetics. I unohistoche ic l st ins i enti y the ly phoi ggreg te s re c-

Proly phocytic leuke i is ch r cterize by l rge tive n the interstiti l inf ltr te s -LGLs. He toxylin n eosin, 20×

nu ber o s ll ly phocytes with sc nt cytopl s n gnif c tion. (Fro Sun . Flow Cytometry, Immunohistochemistry,
and Molecular Genetics or Hematologic Neoplasms, 2n e ,
the i ture e tures o proly phocytes in the periph- Phil elphi , PA: Lippincott Willi s & Wilkins, 2012.)

er l bloo . Cells o -PLL o en h ve pro inent nucleolus

e iu or s ll in size with convolute nucle r outlines.

-PLL in peripher l bloo exhibits s ll to e iu , roun Prognosis and Treatm ent

or irregul r nuclei rese bling Séz ry cells. Pro inent nucle- -cell PLL is typic lly ggressive, but subset o p tients y

oli ppe r only in s ll proportion o c ses, but cytopl s- exhibit n in olent ph se o v ri ble length. -PLL p tients

ic blebbing is co on. h ve e i n surviv l o 7.5 onths.

Leukocytosis c n excee 100 × 10 /L. Proly phocytes First-line ther py or p tients with -PLL is le tuzu b.
9
ust excee 55% o ly phoi cells in the peripher l bloo . Eligible p tients y be consi ere or llogenic bone r-

In c ses o -cell PLL, the i unophenotypes re CD2+, row tr nspl nt tion.

CD3+, CD5+, CD7 +(very strong), n CD52 +(very strong).

Most p tients re CD4+. Neg tive results re observe or Sézary Syndrome and Mycosis Fungoides

, CD1 , n CD25. Cytogenetic bnor lities inclu e

Inv 14, t(14;14), t(x;14), n Iso 8q co plex. Molecul r T e leuke ic ph se o cut neous -cell ly pho (C CL)

genetics reve l ut tions CL1, M CP 1, A M JAK3, n is ycosis ungoi es (MF) (Fig. 22.6). It is the ost co on

S A 5b. v ri nt o C CLs.

Genetic Features and Epstein-

TABLE 22.5 Barr Virus Status in Selected

T-Cell Neoplasms

Genetic

Type of Neoplasm Abnormality EBV Status

T-PLL inv 14, trisomy 8q −

T-LGL None known −

Mycosis fungoides/ None known −

Sézary syndrome FIGURE 22.6 Mycosis ungoi es (cut neous -cell ly pho ).

EBV Epstein-Barr virus; T-PLL, T-cell prolymphocytic leukemia/lymphoma; Lesions h ve ch r cteristic “s u gy,” poorly e ine p tches
,
T-LGL, T-cell large granular lymphocytic leukemia. n pl ques in typic l loc tion. (Reprinte ro Goo he rt
HP. Goodheart’s Photoguide o Common Skin Disorders, 2n e ,
Source: Handin RI, et al. (eds.). Blood: Principles and Practice of

Hematology, 2nd ed, Philadelphia, PA: Lippincott Williams & Wilkins, 2003. Phil elphi , PA: Lippincott Willi s & Wilkins, 2003, with
per ission.)