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Objective To describe the management and outcomes of Fanconi anemia (FA) patients with head and neck squamous cell carcinoma. Study Design Cohort study. Methods Demographic information, prognostic factors, therapeutic management, and survival outcomes for FA patients enrolled in the International Fanconi Anemia Registry (IFAR) who developed head and neck squamous cell carcinoma (HNSCC) were analyzed. Results 35 FA patients were diagnosed with HNSCC at a mean age of 32 years. The most common site of primary cancer was the oral cavity (26/35, 74%). Thirty patients underwent surgical resection of the cancer. Sixteen patients received radiation therapy with an average radiation dose of 5050 cGy. The most common toxicities were high-grade mucositis (9/16, 56%), hematologic abnormalities (8/16, 50%), and dysphagia (8/16, 50%). Three patients received conventional chemotherapy and had significant complications while three patients who received targeted chemotherapy with cetuximab had fewer toxicities. The 5-year overall survival rate was 39% with a cause-specific survival rate of 47%. Conclusions Fanconi anemia patients have a high risk of developing aggressive HNSCC at an early age. FA patients can tolerate complex ablative and reconstructive surgeries, but careful post-operative care is required to reduce morbidity. The treatment of FA-associated HNSCC is difficult secondary to the poor tolerance of radiation and chemotherapy. However, radiation should be used for high-risk cancers because of the poor survival in these patients.

The pre-engraftment syndrome (PES) after cord blood (CB) transplantation (CBT) is poorly characterized. Therefore, we reviewed 52 consecutive double unit CBT recipients treated for high-risk hematological malignancies. PES was defined as unexplained fever >38.3°C (101F) not associated with infection and unresponsive to antimicrobials, and/or unexplained rash occurring before or at neutrophil recovery. CBT recipients (median 38 years, range 3-66) received either myeloablative (n=36) or non-myeloablative (n=16) conditioning. Sixteen patients (31%) fulfilled PES criteria: 15 with fever [onset median 39°C (102.2F)] with 13 also having rash, and one with rash alone. The median onset was 9 days (range 5-12) post-transplant (a median of 14 days before neutrophil recovery). Sixteen patients received IV methylprednisolone (MP) (14 PES, 2 with infection and possible PES; median dose 1 mg/kg, median duration 3 days) with 15/16 (94%) having fever resolution in ≤24 hours. Recurrent PES (n=3) resolved with retreatment. There was no association between the development of PES and the likelihood of sustained donor engraftment, the speed of neutrophil recovery, grade II-IV acute graft-versus-host disease (aGVHD), day 180 transplant-related mortality, or survival. PES is common after CBT, precedes neutrophil recovery, is distinct from and does not predict for aGVHD, and responds promptly to short course corticosteroids.

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