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CUTANEOUS LYMPHOMAS + LEUKEMIAS - Coggle Diagram
CUTANEOUS LYMPHOMAS + LEUKEMIAS
T CELL
MYCOSIS FUNGOIDES
Patch stage:
Lymphocytes 'line up along the dermal-epidermal junction (simulates vacuolar interface dermatitis, with a 'lymphocyte in every hole')
Large dark lymphocytes with irregular nuclear contours and perinuclear haloes ('lump of coal on a pillow')
Pautrier's microabscesses
(intraepidermal clusters of atypical lymphocytes, larger than the benign recruited dermal lymphocytes)
Mild band-like infiltrate in the superficial dermis
Sclerosis of the papillary dermis
Eosinophils and necrotic keratinocytes are rarely present
Plaque stage
:
Like patch stage but with denser, band-like infiltrate in the upper dermis
Atypical lymphocytes present in a dermal band
Tumor stage
Dense, nodular lymphocytic infiltrate in the superficial and deep dermis
Many atypical lymphocytes present in the dermal infiltrate
Transformation to large-sized lymphocytes in some cases
Acquisition of CD30 expression in some cases
Loss of epidermotropism with progression
VARIANTS
PAGETOID RETICULOSIS VARIANT
Solitary or multiple patches or plaques on distal extremities
Atypical large lymphocytes extensively infiltrating the epidermis
CD3+
CD43+ CD8- or CD4- CD8+
Small reactive lymphocytes in papillary dermis
FOLLICULOTROPIC VARIANT
Atypical lymphocytic infiltrate in the follicular epithelium
Basaloid induction and hyperplasia of follicular epithelium
Eosinophils common
Follicular mucinosis
(pools of mucin in the follicular epithelium)
Epidermis usually spared
CD3+ CD4+ CD8- in most cases
GRANULOMATOUS SLACK SKIN
An extremely rare variant of MF with a slowly progressive clinical course
Pendulous folds in intertriginous regions
Preceded by insidious onset of patches, papules and plaques
Massive dermal and subcutaneous infiltrate +/- epidermal involvement
Small T lymphocytes with epidermotropism and mild cytologic atypia
Huge multinucleate giant cells with numerous nuclei, often in wreath-like arrangement
Phagocytosis of lymphocytes by multinucleate cells
Dermal edema or fibrosis
Loss of dermal elastic tissue fibres
CD3+ CD4+ CD8- immunophenotype
SEZARY SYNDROME
Although previously thought to be a leukemic variant of MF, now considered to originate from a different subset of T cells (central memory T cell vs skin resident memory T cell in MF)
Rapid clinical course and poor prognosis
Erythroderma with generalized pruritus
Palmoplantar keratoderma
Generalized lymphadenopathy
Peripheral blood Sezary cell count of >/= 1000 cells/microlitre
Peripheral blood lymphocytes with aberrant phenotype or CD4/8 ratio >10
Clonal rearrangement of T-cell receptor genes in blood and/or skin
Histopathology like MF or may show only non-specific dermatitis
ADULT T-CELL LEUKEMIA/LYMPHOMA (ATCLL)
Well-established viral etiology:
HTLV-1
(transmitted by sexual contact, vertical transmission and blood transfusion)
Endemic in Japan, Caribbean, southeastern USA and Central Africa
Can occur as an acute or smouldering disease
Hypercalcemia
Osteolytic bone lesions
Organomegaly
Lymphadenopathy
Dermal and/or subcutaneous lymphoid infiltrates
T cells with multilobed nuclei
Epidermotropism in some cases
CD3+ CD4+ CD8- CD25+ neoplastic cell population in blood, nodes, skin
Peripheral blood
flower cells
with multilobed nuclei
Clonal integration of the HTLV-1 genome within neoplastic cells
Clonal rearrangement of T-cell receptor genes
PRIMARY CUTANEOUS CD30+ LYMPHOPROLIFERATIVE DISORDERS
LYMPHOMATOID PAPULOSIS
Patients present with crops of ulcerated nodules and papules most frequently involving the trunk and limbs
Lesions regress spontaneously, while new lesions erupt at other sites
Type A
Wedge-shaped dermal infiltrate with mixed population of cells
CD30+
Reed-Sternberg-like cells
with large nuclei, prominent nucleoli and abundant cytoplasm
Neutrophils, eosinophils and small lymphocytes in backgroung
Type B
Epidermotropic infiltrate of CD3+ small lymphocytes, often CD30-
Type C
Diffuse sheets of CD30+
Reed Sternberg-like cells
in dermis
Type D
Markedly epidermotropic CD8+ CD30+ lymphocytes, often TIA-1+ or granzyme B+
Type E
Angioinvasive CD30+ BetaF1+ lymphocytes, often CD8+ and/or TIA-1+
PRIMARY CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA
Sheets of large Reed-Sternberg-like cells in dermis +- subcutaneous fat
Reactive small lymphocytes, histiocytes and eosinophils in the background
CD30 expression by >75% of large cells in infiltrate
Most often presents as solitary or localized tumors or nodules
Spontaneous regression occasionally occurs
Prognosis favourable (>90% at 5-years)
SUBCUTANEOUS PANNICULITIS-LIKE T-CELL LYMPHOMA
Lace-like rimming of infiltration in the fat
Lymphoid cells rimming the individual adipocytes
No significant involvement of dermis or epidermis
Variable cytologic atypia, minimal to marked
Cytophagocytosis:
Beanbag cells
: macrophages filled with karyorrhectic debris
CD8+, CD4-, alpha/beta TCR (BF1)+, gamma/delta TCR -
Cytotoxic phenotype: granzyme B+ TIA-1+ perforin+
EXTRANODAL NK/T CELL LYMPHOMA, NASAL TYPE
Dense lymphoid infiltrates in the dermis, subcutaneous fat +/- epidermis
Angiocentricity, angiodestruction, and zonal necrosis
CD2+, CD3epsilon+, usually CD56+ (NK-cell marker)
CD3 expression variable
Cytotoxic phenotype: granzyme B+ TIA-1+ perforin+
EBV detected within lymphoma cells by in situ hybridization
PRIMARY CUTANEOUS PERIPHERAL T-CELL LYMPHOMA
PRIMARY CUTANEOUS AGGRESIVE EPIDERMOTROPIC CD8+ CYTOTOXIC T-CELL LYMPHOMA
Band-like, lichenoid lymphoid infiltrate with marked epidermotropism
Beta F1+ CD3+ CD8+
Cytotoxic phenotype: granzyme B+ TIA-1+ perforin +
Usually presents with patches, papules, nodules or tumors. Ulceration and hemorrhage are frequent. Clinical course is rapidly progressive
CUTANEOUS GAMMA-DELTA T-CELL LYMPHOMA
Location of infiltrate: subcutaneous, dermal, epidermal
TCR gamma-delta+ Beta F1- CD3+ CD56+ CD4-
Cytotoxic phenotype: granzyme B+ TIA-1+ perforin+
Clonal rearrangement of TCR genes
PRIMARY CUTANEOUS CD4+ SMALL/MEDIUM T-CELL LYMPHOMA
Dermal infiltrate +/- involvement of subcutaneous fat and epidermis
CD3+ CD4+ CD8- CD30-
Aberrant immunophenotype with loss of some T-cell markers
Clonal rearrangement of TCR genes
HYDROA VACCINIFORME-LIKE LYMPHOMA
Asian, Mexican, Central and South American children
Ulcerative papulovesicles with scarring
Face and extremities
Hypersensitivity to mosquito bites
Angiocentric infiltrates of small or medium-sized lymphocytes
EBV detected within tumor cells by ISH
TIA-1+ CD2+ CD8 and CD56 variable
PRECURSOR HEMATOLOGIC NEOPLASM
BLASTIC PLASMACYTOID DENDRITIC CELL NEOPLASM
Monomorphous or polymorphous infiltrate in the dermis or subcutis
No epidermotropism
No angioinvasion or necrosis
CD4+ CD56+ CD123+ CD3- MPO-
Cytotoxic markers negative
No clonal rearrangement of TCR genes
Negative ISH for EBV
MYELOID LEUKEMIA
Diffuse interstitial, perivascular and periadnexal infiltrate in dermis and subcutis
No epidermotropism
Grenz zone separates the infiltrate from the epidermis
Single filing of cells splaying dermal collagen
Mononuclear cells (blasts) ranging from large to small
Prominent nucleoli (variable)
Eosinophilic cytoplasm (variable)
Scattered bilobed cells resembling neutrophilic 'bands' sometimes present
CD3- CD20- CD43+
Lysozyme+ MPO+ chloracetate esterase+
Cutaneous involvement more common in AML than CML
Patients present with papules, nodules, plaques, purpura or ulcers (involvement of gingivae common)
B CELL
CUTANEOUS B-CELL LYMPHOPROLIFERATIVE DISORDERS
PRIMARY CUTANEOUS MARGINAL ZONE LYMPHOMA
PRIMARY CUTANEOUS FOLLICLE CENTRE CELL LYMPHOMA
CUTANEOUS DIFFUSE LARGE B-CELL LYMPHOMA, LEG TYPE
CUTANEOUS DIFFUSE LARGE B-CELL LYMPHOMA, OTHER THAN LEG TYPE
LYMPHOID GRANULOMATOSIS
CHRONIC LYMPHOCYTIC LEUKEMIA/SMALL LYMPHOCYTIC LYMPHOMA
MANTLE CELL LYMPHOMA
BURKITT'S LYMPHOMA
B-CELL LYMPHOBLASTICV LYMPHOMA/LEUKEMIA