Primary Cutaneous Anaplastic Large Cell Lymphoma: A Comprehensive Approach to Effective Treatment

When a 52-year-old male patient first noticed a small, raised skin lesion on his leg about a year ago, he didn't think much of it. However, as the lesion grew larger, became painful, and started causing persistent itching that disrupted his sleep and daily activities, he decided to seek medical attention. After thorough evaluation at our institution—Sun Yat-sen University Cancer Center—we diagnosed him with primary cutaneous CD30-positive anaplastic large cell lymphoma (PCALCL), a rare but treatable form of non-Hodgkin lymphoma originating in the skin.

This case highlights how early detection, accurate diagnosis, and timely intervention can significantly improve outcomes for patients with cutaneous lymphomas. With a combination of low-dose methotrexate therapy and multi-agent chemotherapy, this patient experienced marked symptom relief and disease control. Below is a detailed overview of his journey from initial presentation to successful treatment and ongoing management.

Patient Profile & Clinical Background

Age: 52 yearsGender: MaleDiagnosis: Primary Cutaneous Anaplastic Large Cell Lymphoma (CD30-positive)Hospital: Sun Yat-sen University Cancer CenterDate of Admission: September 2022Treatment Plan: Combination therapy including intravenous methotrexate, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisoloneTreatment Duration: Initial inpatient stay of 5 days followed by continued outpatient chemotherapy cyclesOutcome: Significant improvement in symptoms; stable clinical condition with no signs of progression

Initial Evaluation and Diagnosis

The patient presented with a progressively enlarging, painful, and pruritic skin lesion on his right lower extremity. On physical examination, a well-demarcated, infiltrative plaque measuring approximately 6 cm in diameter was observed. The lesion appeared dark red, slightly elevated above the surrounding skin, and showed signs of ulceration with serous discharge—features highly suggestive of a cutaneous neoplastic process such as lymphoma.

Given the atypical appearance and progressive nature of the lesion, we recommended a skin biopsy for definitive diagnosis. Laboratory tests, including complete blood count and metabolic panel, were within normal limits, which is common in early-stage PCALCL. Histopathological analysis revealed diffuse infiltration of large, pleomorphic cells into the dermis and subcutaneous tissue. Mitotic figures were frequent, indicating active cell division. Immunohistochemistry confirmed strong CD30 positivity, supporting the diagnosis of primary cutaneous anaplastic large cell lymphoma.

Why Early Detection Matters

Early recognition of unusual skin changes is crucial. Unlike more aggressive systemic lymphomas, PCALCL often has an indolent course and responds well to localized or systemic treatments when caught early. Delayed diagnosis, however, may lead to extensive skin involvement or extracutaneous spread, complicating treatment strategies.

Personalized Treatment Strategy

After discussing all available options—including radiation therapy, single-agent chemotherapy, and combination regimens—we opted for a tailored approach using low-dose methotrexate combined with a modified CHOP-like regimen (cyclophosphamide, doxorubicin, vincristine, and corticosteroid). This strategy balances efficacy with reduced toxicity, especially important for middle-aged patients aiming to maintain quality of life during treatment.

The patient received intravenous infusions over five consecutive days during his initial hospitalization. Methotrexate was administered weekly thereafter, while the other agents were given in cyclic fashion under close monitoring. He tolerated the protocol well, reporting only mild fatigue without severe nausea, infection, or neuropathy.

How Combination Therapy Works

Methotrexate interferes with DNA synthesis in rapidly dividing tumor cells. Cyclophosphamide and doxorubicin are potent cytotoxic agents targeting multiple pathways in cancer cell proliferation. Vincristine disrupts microtubule formation, halting mitosis. Prednisolone, a corticosteroid, reduces inflammation and enhances the apoptotic effect on malignant lymphocytes. Together, these drugs create a synergistic anti-tumor response while minimizing resistance development.

Positive Response and Follow-Up Outcomes

Five days after initiation of treatment, the patient's vital signs remained stable, and there were no acute adverse reactions. Due to his improved comfort level and expressed desire to continue recovery at home, he was discharged with clear instructions for follow-up care. Subsequent outpatient visits confirmed continued improvement: the skin lesion began to regress, pain and itching diminished significantly, and he reported better sleep and overall well-being.

Follow-up imaging and laboratory assessments showed no evidence of disease progression. Repeat biopsies are not routinely required unless new lesions appear, but regular clinical evaluations remain essential. As of the latest check-up, the patient continues with maintenance therapy and remains in good health.

Key Recommendations for Patients Undergoing Chemotherapy

Managing side effects and maintaining general health during treatment is critical. Here are several practical tips I shared with the patient and his family:

• Monitor Blood Counts Regularly: Even if you feel fine, routine blood tests (CBC, liver and kidney function) help detect potential complications early.
• Address Peripheral Neuropathy Promptly: If numbness, tingling, or cold sensitivity occurs in hands or feet—common with vincristine—try warm compresses, heated socks, or foot soaks. Report symptoms immediately to adjust dosing if needed.
• Maintain a Soft, Nutrient-Rich Diet: Low platelet counts increase bleeding risk. Choose soft foods like oatmeal, scrambled eggs, mashed potatoes, and smoothies to avoid oral trauma.
• Protect Skin Integrity: Keep the affected area clean and moisturized. Avoid scratching or using harsh soaps. Use fragrance-free emollients to prevent dryness and cracking.
• Stay Hydrated and Rest Well: Proper hydration supports drug metabolism and helps flush toxins. Prioritize rest to support immune recovery between cycles.

Insights from Clinical Practice

While the exact cause of CD30-positive cutaneous anaplastic large cell lymphoma remains unclear, it tends to affect middle-aged and older adults, particularly men. There is some evidence linking chronic antigen stimulation or environmental exposures—such as industrial chemicals or prolonged UV radiation—to increased risk, though no direct causative agent has been identified.

One key takeaway from this case is the importance of vigilance. Many patients dismiss skin abnormalities as harmless rashes, insect bites, or minor infections. But any persistent, growing, or symptomatic skin lesion lasting beyond a few weeks warrants professional evaluation. Dermatoscopy and biopsy can differentiate benign conditions from malignancies like PCALCL, which often mimic other dermatological diseases.

Prevention and Proactive Health Screening

Although there's no guaranteed way to prevent PCALCL, individuals—especially those over 50 or with long-term exposure to chemical agents—should consider annual dermatologic screenings. Routine full-body skin exams can catch early signs of cutaneous lymphoma or other skin cancers. Additionally, prompt consultation upon noticing unexplained nodules, plaques, or ulcers can lead to earlier interventions and better prognoses.

In conclusion, primary cutaneous anaplastic large cell lymphoma, while rare, is a manageable condition with modern therapeutic approaches. Through personalized combination therapy and attentive supportive care, patients can achieve excellent clinical responses and maintain a high quality of life. Awareness, early diagnosis, and adherence to treatment plans are the cornerstones of success.