Mature T and NK cell neoplasm
2008
 T-cell prolymphocytic leukemia
 T-cell large granular lymphocytic leukemia
 Chronic lymphoproliferative disorder of
NK cells
 Aggressive NK cell leukemia
 EBV+ T-cell Lymphoproliferative
disorder of childhood
 Adult T-cell leukemia/lymphoma
 Extranodal NK/T-cell lymphoma, nasal
type
2016
 T-cell prolymphocytic leukemia
 T-cell large granular lymphocytic leukemia
 Chronic lymphoproliferative disorder of
NK cells
 Aggressive NK cell leukemia
 Systemic EBV+ T-cell Lymphoma of
childhood*
 Hydroa vacciniforme-like
lymphoproliferative disorder*
 Adult T-cell leukemia/lymphoma
 Extranodal NK/T-cell lymphoma, nasal
type
Systemic EBV1 T-cell lymphoma of childhood

 Name changed from lymphoproliferative disorder to lymphoma due to its fulminant

clinical course : die within days to weeks of diagnosis.

 Common in Asians (Japan, Taiwan)

 Associated with a haemophagocytic syndrome.

 M/E : small T cells ( can be medium to large as well) involving Liver ,spleen,
lymph node, bone marrow

 CD2+, CD3+, C D 5 6 - , CD 8 +, CD 4 +/-

Hydroa vacciniforme–like lymphoproliferative
Disorder

 Lymphoproliferative disorder due to its clinical course: 10-15 yrs of

cutaneous involvement before progressing to systemic involvement.

 Children , sun exposed areas, papulo-vesicular rashes

• Resistant to conventional chemotherapy, and treated patients often

die of infectious complications.

• Conservative approach recommended in indolent cases.

2008 2016
 Enteropathy-associated T-cell lymphoma  Enteropathy-associated T-cell lymphoma

 Monomorphic epitheliotropic intestinal

T-cell lymphoma*
 Indolent T-cell lymphoproliferative
disorder of the GI tract *
 Hepatosplenic T-cell lymphoma
 Subcutaneous panniculitis- like T-cell
 Hepatosplenic T-cell lymphoma lymphoma
 Subcutaneous panniculitis- like T-cell  Mycosis fungoides
lymphoma  Sézary syndrome
 Mycosis fungoides
 Sézary syndrome
Enteropathy-associated T-cell lymphoma (EATL)
• Diagnosis only to be used for cases formerly known as type I
EATL, typically associated with celiac disease.
• Poor prognosis.

Pleomorphic
intestinal infiltrate
extends into the
epithelium.

CD56 -
Monomorphic epitheliotropic intestinal T-cell
lymphoma

• Formerly type II EATL; segregated from type I EATL and given a

new name due to its distinctive nature and lack of association
with celiac disease.

The monotonous
intestinal infiltrate
(strong CD56 +) is
very epitheliotropic.
2008
 Primary cutaneous CD30 positive T-cell
lymphoproliferative disorders
-Lymphomatoid papulosis
-Primary cutaneous anaplastic large cell
lymphoma
 Primary cutaneous gamma-delta T-cell
lymphoma
 Primary cutaneous CD8 positive aggressive
epidermotropic cytotoxic T-cell lymphoma
 Primary cutaneous CD4 positive
small/medium T cell lymphoma
2016
 Primary cutaneous CD30 positive T-cell
lymphoproliferative disorders
-Lymphomatoid papulosis
-Primary cutaneous anaplastic large cell
lymphoma
 Primary cutaneous gamma-delta T-cell
lymphoma
 Primary cutaneous CD8 positive aggressive
epidermotropic cytotoxic T-cell lymphoma
 Primary cutaneous acral CD8+ T-cell
lymphoma*
 Primary cutaneous CD4 positive
small/medium T-cell lymphoproliferative
disorder
Primary cutaneous acral CD8+ T-cell lymphoma

• New indolent provisional entity, originally

described as originating in the ear.

Primary cutaneous acral CD8 + TCL. (C) Nodule on the ear. (D) There is a
diffuse monotonous infiltrate of CD8 + T cells.
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

• No longer to be diagnosed as an overt lymphoma due to limited clinical

risk, localized disease, and similarity to clonal drug reaction.

• Dense, diffuse, or nodular infiltrates within the dermis, with a tendency to

infiltrate the subcutis.

• CD4+ T cells are admixed with small reactive CD8+ T cells, B cells, plasma
cells, and histiocytes.
• If epidermotropism is conspicuous

• D/D: Mycosis fungoides.

• excellent prognosis.

• lntralesional steroids, surgical excision, and radiotherapy are

preferred modes of treatment
2008
 Peripheral T-cell lymphoma, NOS
 Angioimmunoblastic T-cell
lymphoma
 Anaplastic large cell lymphoma,
ALK positive
 Anaplastic large cell lymphoma,
ALK negative
2016
 Peripheral T-cell lymphoma, NOS
 Angioimmunoblastic T-cell
lymphoma
 Follicular T-cell lymphoma*
 Nodal peripheral T-cell lymphoma
with TFH phenotype*
 Anaplastic large cell lymphoma,
ALK positive
 Anaplastic large cell lymphoma,
ALK negative (definite)
 Breast implant-associated anaplastic
large cell lymphoma
 Nodal T-cell lymphomas with T follicular helper (TFH)
phenotype

• A subset of the peripheral T-cell lymphomas NOS, having T follicular

helper (TFH) cell phenotype.

• Now definitive entity

• TFH phenotype: at least 2 or 3 of TFH-related antigens: CD279/PD1,

CD10, BCL6, CXCL13, ICOS, SAP, CCR5.

• Better differentiation between AITL and PTCL NOS

 Follicular T Cell Lymphoma
• Neoplasm of T-follicular helper (TFH) cells, with a predominantly
follicular growth pattern .

• Lacking characteristic feature of angioimmunoblastic T-cell lymphoma

(AITL) such as proliferation of high endothelial venules or
extrafollicular follicular dendritic cells

• Very rare (incidence unknown)

• But both entities have poor prognosis

ALK-negative anaplastic large cell
lymphoma
• T-cell lymphoma consisting of lymphoid cells that are usually
large and have abundant cytoplasm and pleomorphic, often
horseshoe-shaped nuclei.
• Poor prognosis than ALK + ALCL.

CD 30 + membranous
Breast implant–associated anaplastic large cell
lymphoma
• New provisional entity distinguished from other ALK- ALCL;
noninvasive disease associated with excellent outcome.

The seroma cavity demonstrates

numerous very large anaplastic-
appearing lymphoid cells
• Both saline and silicone filled
implants

• Median interval from implant

to the lymphoma 10 yr

• Treatment: removal of the

implant and capsule.

• If invasion through the

capsule: systemic
chemotherapy
 POST-TRANSPLANT LYMPHOPROLIFERATIVE DISORDERS (PTLD)

2008 2016
 Plasmacytic hyperplasia PTLD  Plasmacytic hyperplasia PTLD
 Infectious mononucleosis PTLD  Infectious mononucleosis PTLD
 Florid follicular hyperplasia PTLD*

 Polymorphic PTLD  Polymorphic PTLD

 Monomorphic PTLD (B- and T/NK-  Monomorphic PTLD (B- and T/NK-
cell types) cell types)
 Classical Hodgkin lymphoma PTLD  Classical Hodgkin lymphoma PTLD
HISTIOCYTIC AND DENDRITIC CELL NEOPLASMS
2008
 Histiocytic sarcoma
 Langerhans cell histiocytosis
 Langerhans cell sarcoma
 Indeterminate dendritic cell tumour
 Interdigitating dendritic cell sarcoma
 Follicular dendritic cell sarcoma
 Fibroblastic reticular cell tumour
 Disseminated juvenile
xanthogranuloma
2016
 Histiocytic sarcoma
 Langerhans cell histiocytosis
 Langerhans cell sarcoma
 Indeterminate dendritic cell tumour
 Interdigitating dendritic cell sarcoma
 Follicular dendritic cell sarcoma
 Fibroblastic reticular cell tumour
 Disseminated juvenile
xanthogranuloma
 Erdheim Chester disease
Erdheim-Chester disease (ECD)

• Clonal systemic proliferation of histiocytes, commonly having a foamy

(xanthomatous) component, and containing Touton giant cells.

• < 1000 cases have been reported.

• mean patient age : 55- 60 years

• Virtually any organ or tissue can be infiltrated.

• Skeletal involvement occurs in > 95% of cases. (lytic and sclerotic lesions)
• patients with CNS disease or multisystemic disease have a worse
outcome

• mutated BRAF

• CD 68 + histiocytes

• Neg for S100, CD1a and langerin.

HODGKIN LYMPHOMA
2008
• Nodular lymphocyte predominant
Hodgkin lymphoma
• Classical Hodgkin lymphoma
Nodular sclerosis classical HL
Lymphocyte-rich classical HL
Mixed cellularity classical HL
Lymphocyte-depleted classical HL
2016
• Nodular lymphocyte predominant
Hodgkin lymphoma
• Classical Hodgkin lymphoma
Nodular sclerosis classical HL
Lymphocyte-rich classical HL
Mixed cellularity classical HL
Lymphocyte-depleted classical HL
Thank you !!