Mycosis Fungoides and Sézary Syndrome
Definition
• Constitutes 1 of the most frequent primary cutaneous T-cell lymphomas
• Sézary syndrome is an erythrodermic leukemic variant of MF, but is now classified as a separate form of cutaneous lymphoma itself. Denotes combination of skin & peripheral blood involvement (>20% tumor lymphocytes or 1000/mm3 absolute cells).
• Postulated etiologies include environmental/occupational exposures, viral (HTLV-1 & CMV), histocompatibility antigen associations, & chromosomal abnormalities
• The tumor cells of MF infiltrate the epidermis & circulate in the blood, & these cells express T-cell–associated antigens (CD2μ+μ, CD3μ+μ, *CD4μ+μ, CD5μ+μ, & CD7μ+μ)
• Extracutaneous involvement can occur, most commonly in lung, spleen, liver, GI tract
History
• Median age 55–60 y/o w/ M:F of 2:1
• Indolent evolving rash w/ intense pruritus
Physical Findings
• Often begins w/ an indolent, persistent cutaneous eruption of asymmetrical, irregular, sun-protected erythematous scaly patches or plaques (often resembles atopic dermatitis or psoriasis) → generalized plaques → ulcerated or exophytic tumors
• May also present w/ generalized erythroderma w/ intense pruritus
• There may be prominent skin atrophy w/ hyperpigmentation & telangiectasias
• Lymphadenitis
Evaluation
• Histopathologic Dx from skin biopsy, flow cytometry showing expanded pathologic T-cell populations, DNA microarray techniques
Treatment
• Depends on staging & not initiated in ED
Disposition
• Referral to Hematologist/Oncologist when suspected
• Home or admission depending on pt stability, f/u, & local institutional practice
Kaposi’s Sarcoma
Definition
• Kaposi’s sarcoma is a spindle-cell tumor arising from lymphatic endothelium surrounding hyperemic vascular slits giving the lesions their characteristic violaceous appearance
• There are currently 4 recognized variants:
• Classic: Seen in elderly men of Eastern European or Mediterranean origin
• Endemic: Seen in African children & adults
• Immunosuppression/transplant associated: Seen in transplant recipients
• Epidemic, or AIDS associated: Seen in pts w/ HIV w/ AIDS progression & represents an AIDS-defining illness
• HHV-8 (Kaposi’s sarcoma–associated herpesvirus [KSHV]) is central to its pathogenesis; transmitted via sexual contact, mucous membranes, maternal–fetal transmission
• RFs: Endemic country, transplant recipient or on immunosuppression therapy, AIDS, high-risk sexual practices (multiple partners, unprotected sex, MSM, etc.)
History
• Pts typically have 1 or more of the above RFs & present w/ development of an indolent, nonpruritic exanthem described below
• Extracutaneous manifestations may include N/V, dysphagia, UGIB, cough, SOB, hemoptysis, among others
Physical Findings
• Multiple firm, purple-blue or reddish-brown (violaceous) patches & plaques → nodules, typically on hands & feet & progress up the arms & legs
• May also involve oral mucosa, lymph nodes, & visceral organs (GI & pulmonary)
Evaluation
• Histopathologic Dx from skin biopsy/bronchoscopy/EGD depending on location, PCR, & serologic assays to detect KSHV Abs
• Suspected cases should include HIV testing (CD4μ+μ count & viral load)
• CXR
Treatment
• Requires specialist eval, but may include excisional biopsy, XRT, chemotherapeutics, HAART in HIV-AIDS pts
Disposition
• Referral to Hematologist/Oncologist when suspected
• Referral to infectious dz specialist when in the context of HIV
• Home or admission depending on pt stability, f/u, & local institutional practice
Cutaneous Graft versus Host Disease (GVHD)
Background
• Allogeneic hematopoietic stem cell transplant (HSCT) has become an important tx modality for the tx of various hematologic malignancies as well as for nonmalignant stem cell disorders (ie, aplastic anemia), genetic dzs (ie, SCID), & solid tumors (ie, renal cell carcinoma).
• These transplanted stem cells are used from a pt’s own cells (autologous), cells of a twin (syngeneic), or related/unrelated donor (allogeneic) & can be collected from bone marrow, peripheral blood, or umbilical cord blood
• GVHD develops in ∼50% of all pts undergoing allogeneic HSCT
Definition
• GVHD is a common cause of morbidity & mortality in recipients of allogeneic HSCT
• Occurs when donor T cells are clonally expanded in an antigen-specific manner after recognition of nonself human leukocyte antigen (HLA) expressed on the surface of cell in a host that is immunocompromised. These cells primarily attach epithelia of rapidly proliferating tissues such as skin, liver, & GI tract.
• Can be acute (<100 d) or chronic (>100 d) after allogeneic HSCT
History
• Acute cutaneous GVHD presents w/ a skin rash that usually occurs w/i 14–42 d after transplantation
• The skin is usually the 1st target organ affected before the liver & GI tract
• Presentation may be a/w preventive therapy noncompliance
Physical Findings
• Symmetrical erythematous morbilliform or maculopapular eruption involving upper back, lateral neck, palms, soles, & cheeks; occasionally becomes generalized
• Severe dz can progress to erythroderma, bullae formation, & desquamation
• Stage I: 25% TBSA
• Stage II: 25–50% TBSA
• Stage III: 50% TBSA up to erythroderma
• Stage IV: Erythroderma w/ bullae
• Chronic GVHD lesions: Skin dryness, ichthyosis, psoriasiform, pityriasis rosea like → lichen planus like (violaceous lichenoid papules & plaques on dorsal hands/forearms and trunk) & sclerodermoid (plaques of dermal sclerosis)
• Mucous membranes, conjunctiva, hair, & nails may be affected
• A/w high fever, cholestatic hepatitis & intestinal sxs
Evaluation
• CBC, Chem 7, LFTs (bilirubin used for grading)
• GVHD grading incorporates TBSA skin involvement, degree of cholestatic hepatitis, & volume of diarrhea
• Histopathologic Dx from skin biopsy
Treatment
• Requires specialist eval & consultation
• Cyclosporine or tacrolimus used for the prevention w/ or w/o MTX, T-cell depletion, & corticosteroids
• Acute Stage I–II, topical high-potency corticosteroids or tacrolimus
• Acute Stage III–IV, high doses of systemic corticosteroids (methylprednisolone)
Disposition
• Home or admission depending on stage/grade, pt stability, f/u, & local institutional practice