Hurricane Katrina evacuee develops a persistent rash
Diagnosis hinges on palpation and biopsy
To diagnose CTCL, you need to palpate for enlarged lymph nodes and perform a full-thickness punch biopsy of the lesion. If the biopsy is negative and the rash persists, take another biopsy—results can be falsely negative at the beginning stages of the disease.
There are no guidelines on which studies should be used for staging CTCL (TABLE), but some sources recommend that if lymph node involvement is suspected on physical exam, lymph node biopsies should be done in addition to a chest radiograph.3 In more advanced stages, consider a computed tomography (CT) scan of the abdomen and pelvis. A recent study concluded that CT and positron-emission tomography (PET) scans used together were more sensitive in staging, but this may not be cost-effective.4
Late-stage mycosis fungoides is usually associated with immunocompromise. Therefore, HIV testing should be performed in all patients with CTCL. Other important laboratory studies include complete blood count—with differential—as well as peripheral smear looking for Sézary cells, lactic dehydrogenase (LDH), liver function tests, and uric acid.
TABLE
Staging for cutaneous T-cell lymphoma based on the Tumor, Node, Metastasis (TNM) system
| STAGE | TNM STAGING | RECOMMENDED TREATMENTS |
|---|---|---|
| IA | T1, N0, M0: Patch or plaque <10% body surface area | Topical high-potency steroids, PUVA, topical nitrogen |
| IB | T2, N0, M0: Patch or plaque >10% body surface area | mustard, carmustine, or bexarotene |
| IIA | T1–2, N1, M0: Patch or plaque with palpable but pathologically normal lymph node | Same as Stage I; if refractory, use total skin electron beam therapy |
| IIB | T3, N0–1, M0: Tumor/nodule | |
| IIIA | T4, N0, M0:Generalized erythroderma | |
| IIIB | T4, N1, M0: Erythroderma and palpable but pathologically normal lymph node | Chemotherapy or photophoresis, refer to medical oncologist, radiation oncologist, and dermatologist |
| IVA | T1–4, N2–3, M0: Pathological lymph node | |
| IVB | T1–4, N0–3, M1: Visceral (M1) or blood involvement | |
| T: 0–4=indicates size or direct extent of the primary tumor | ||
| N: 0=tumor cells absent from regional lymph nodes; 1=tumor cells spread to closest or small number of regional lymph nodes; 3=tumor cells spread to most distant or numerous regional lymph nodes | ||
| M: 0=no distant metastasis; 1=metastasis to distant organs (beyond regional lymph nodes) | ||