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OP38 – 3035: Incidence of and risk factors for neurological complications of cardiac bypass surgery in children with congenital heart disease
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Rare but deadly: A case of primary cutaneous gamma-delta T-cell lymphoma Sana Zahiruddin, MD, University of Texas Health Sciences Center at Houston, Houston, TX, United States; Madeleine Duvic, MD, The University of Texas MD Anderson Cancer Center, Houston, TX, United States
Simultaneous diagnosis of cutaneous T-cell lymphoma, chronic lymphocytic leukemia, and acute myelogenous leukemia: Treatment and management challenges Michelle Cheng, MD, University of Pittsburgh Medical Center, Pittsburgh, PA, United States; William Johnson, DO, University of Pittsburgh Medical Center, Pittsburgh, PA, United States; Larisa Geskin, MD, Columbia University Medical Center, New York, NY, United States; Jonhan Ho, MD, University of Pittsburgh Medical Center, Pittsburgh, PA, United States; Lisa Grandinetti, MD, University of Pittsburgh Medical Center, Pittsburgh, PA, United States
Introduction: Primary cutaneous gamma-delta T cell lymphoma (CGD-TCL) is a rare form of cutaneous T cell lymphoma (CTCL) associated with a poor prognosis and recently categorized with peripheral T cell lymphomas of the skin. Case presentation: A 65-year-old Caucasian female presented for evaluation of ‘‘nodules’’ on her lower extremities. In 2012, she developed tender, pruritic nodules in her right calf with additional lesions appearing over the next several months. On examination there were multiple erythematous subcutaneous nodules on her bilateral lower extremities, buttocks, and right upper arm. Staging: Multiple biopsies from lesions showed an atypical T cell lymphoid infiltrate with prominent granulomatous inflammation involving the deep dermis. Most lymphocytes expressed CD3 with a CD4:CD8 ratio of 1:1 with a subpopulation also positive for CD56, TCR gamma, and TIA-1. The interpretation was CGD-TCL with prominent granulomatous inflammation. Bone marrow and nodes showed no evidence of lymphoma. Initial PET/CT scan revealed abnormal uptake in the subcutaneous tissue of the trunk and extremities. ANA and anti-ds DNA were negative. Treatment: The patient initially used clobetasol ointment but progressed. Her lesions were controlled on oral bexarotene 225 mg daily and prednisone 20 mg daily, which was decreased to 10 mg over four months. In January 2014, hydroxychloroquine 200 mg BID was added. As she developed new lesions, confirmed with PET/CT imaging, her prednisone therapy was increased to 40 mg daily. Future treatment options include romidepsin, a CD3-diphtheria fusion protein; praletrexate, a folic acid inhibitor; total body electron beam radiation and allotransplant; or standard chemotherapy. Discussion: This case highlights a rare form of panniculitis-like cutaneous lymphoma, which represents \1% of all CTCL. Autoimmune diseases have been reported in 20% of patients with the alpha-beta phenotype. Our patient has the gamma-delta phenotype, which is associated with a very aggressive clinical course with a five year survival of \10%. Her course has been more indolent with partial response to bexarotene and prednisone. This case highlights new and experimental treatment options for peripheral T-cell lymphomas. Conclusion: Primary CGD-TCL is a rare form of peripheral T-cell lymphoma arising in the skin and may mimic inflammatory panniculitis. Prompt excisional biopsy and recognition is crucial in determining the disease course and treatment. Commercial support: None identified.
Cutaneous T-cell lymphoma (CTCL) is a type of non-Hodgkin lymphoma that involves proliferation of malignant CD4+ T-cells with possible involvement of other organs. CTCL and concurrent B-cell chronic lymphocytic leukemia (CLL) is rare, but has been previously reported in a handful of case reports. Simultaneous diagnosis of primary CTCL, CLL, and acute myeloid leukemia (AML) is a previously undocumented phenomenon that presents unique treatment challenges. Case report: A 77-year-old Caucasian male presented with a 10-week history of a severely pruritic, papular dermatitis on his face, arms, and chest. A punch biopsy revealed folliculotropic mycosis fungoides (MF). An excisional lymph node biopsy of palpable lymph nodes revealed a B-cell neoplasm most consistent with CLL, with no histologic support for a T-cell neoplasm. Further evaluation including a bone marrow biopsy revealed increased blasts and promonocytes of 24%, consistent with AML type M5. Patient declined treatment for CLL and AML. The main issue affecting his quality of life was severe skin pruritus. To select therapy for his MF and AML, the therapeutic choices included retinoids. However, patient was unable to tolerate even low dose bexarotene therapy due to recurrent severe neutropenia, necessitating G-CSF (filgrastim) injections. Extracorporeal photopheresis every 2 weeks brought some improvement in his pruritus. Ultimately, his AML progressed rapidly and the patient passed away. Conclusion: We present this patient diagnosed with consecutive triple primary hematologic malignancies, including CTCL, B-cell CLL, and AML, to highlight the difficulty in treating CTCL in patients with other hematologic malignancies. Bexarotene is a well-established treatment for CTCL; it is also believed to have antileukemic activity and to be well tolerated in nonM3 AML. Therefore, despite using intervention known to be safe in patients with CTCL or AML, it was a challenge to treat his CTCL with his concurrent CLL and AML due to recurring bone marrow suppression. Although multiple studies have investigated these interventions, none address its risks in patients with concurrent disease. The incidence of second malignancies in patients with CTCL is high. Case studies of CLL and other malignancies with CTCL have been reported. However, it is rare to have three hematologic malignancies in the same patient and they pose significant therapeutic challenges confounded by significant bone marrow suppression. Commercial support: None identified.
1660 Severe leonine facies in a case of folliculotropic mycosis fungoides Casey Wang, MD, University of Texas MD Anderson Cancer Center, Houston, TX, United States; Madeleine Duvic, MD, University of Texas MD Anderson Cancer Center, Houston, TX, United States; Bouthaina Dabaja, MD, University of Texas MD Anderson Cancer Center, Houston, TX, United States; Rakshandra Talpur, MD, University of Texas MD Anderson Cancer Center, Houston, TX, United States Introduction: Folliculotropic mycosis fungoides (FMF) is a rare variant of mycosis fungoides (MF) in which atypical T-cells infiltrate and destroy hair follicles and occasionally sweat glands (termed syringotropic). Unlike classic MF, FMF rarely exhibits epidermotropism. Morphologically, FMF is associated with acneiform lesions, pustules, cystic milia-like lesions, and follicular mucinosis. Leonine facies is an uncommon but potentially devastating development, resulting from nodular infiltration of the face. We present a patient with aggressive FMF with large cell transformation who rapidly developed severe leonine facies and was resistant to systemic chemotherapy. Case report: A 67-year-old African American male presented for treatment of severe facial swelling. The patient’s symptoms began 1 year ago with irritation of his chin. A skin biopsy was consistent with mycosis fungoides. He had progressive disease on topical medications, oral bexarotene, 5 cycles of R-CHOP, and 3 cycles of gemcitabine and carboplatin. 2 weeks prior to presentation, the patient developed progressive facial swelling that compromised his eyesight, hearing, and speech. The skin examination was significant for profound leonine facies, large confluent hyperpigmented plaques on his trunk, and scattered annular erosions with scale. A biopsy of his cheek showed a lymphocytic infiltrate of large cells in the dermis with folliculotropism, focal epidermotropism, and CD4:CD8 ratio [ 10. Peripheral blood flow cytometry showed absolute CD4+CD26e cell count of 2032 uL consistent with Sezary syndrome. FNA biopsy of an axillary node showed involvement by large cells. A bone marrow biopsy showed T-cell lymphoma comprising 20% of the marrow cellularity. While admitted, the patient was treated with 2500 cGy of local radiation to his face with improvement. However he developed exfoliation and dryness of his face that resulted in large bleeding fissures. Wound culture showed colonization with MRSA. During his treatment, he developed altered mental status and fevers. He ultimately succumbed to infection.
1944 The mantle cell masquerade Sphoorthi Jinna, MD, MS, University of Connecticut Health Center, Farmington, CT, United States; Jim Whalen, MD, University of Connecticut Health Center, Farmington, CT, United States Background: Mantle cell lymphoma (MCL) is a relatively rare type of non-Hodgkin lymphoma that commonly affects extranodal sites. Lesions typically occur in the gastrointestinal tract or bone marrow, with cutaneous manifestations being relatively uncommon. Clinical presentation can be diverse and is generally a result of metastasis. Given 90% of MCL patients currently present with stage III-IV disease, a high index of suspicion for unique presentations can result in earlier diagnosis. We report a case of an unusual cutaneous manifestation of MCL, namely as a deep facial subcutaneous nodule. Case description: A 76-year-old male with 3-6 month history of multiple painless enlarging subcutaneous nodules on his left cheek presented for evaluation. One nodule was excised for both diagnostic and therapeutic indications. Pathology revealed mantle cell lymphoma and the patient was promptly referred to oncology for further management.
Discussion: Folliculotropic mycosis fungoides is a therapeutic challenge as it is often resistant to standard therapy. The role of radiation in the treatment of leonine facies is unclear. Several studies report that FMF is associated with worse prognosis, comparable to that of classic tumor stage MF. Large cell transformation is also a poor prognostic factor and is associated with more advanced stages of MF.
Discussion: Given mantle cell lymphoma generally presents advanced stage and therefore with a poor prognosis, early detection is of paramount importance, especially in the wake of new systemic therapies. Case reports of cutaneous manifestations of MCL presenting as maculopapular rash or infiltrated plaques have been reported, but we report on the unusual presentation of MCL as a deep facial subcutaneous nodule.
Commercial support: None identified.
Commercial support: None identified.
MAY 2015
J AM ACAD DERMATOL
AB163
1458
Rare but deadly: A case of primary cutaneous gamma-delta T-cell lymphoma Sana Zahiruddin, MD, University of Texas Health Sciences Center at Houston, Houston, TX, United States; Madeleine Duvic, MD, The University of Texas MD Anderson Cancer Center, Houston, TX, United States
Simultaneous diagnosis of cutaneous T-cell lymphoma, chronic lymphocytic leukemia, and acute myelogenous leukemia: Treatment and management challenges Michelle Cheng, MD, University of Pittsburgh Medical Center, Pittsburgh, PA, United States; William Johnson, DO, University of Pittsburgh Medical Center, Pittsburgh, PA, United States; Larisa Geskin, MD, Columbia University Medical Center, New York, NY, United States; Jonhan Ho, MD, University of Pittsburgh Medical Center, Pittsburgh, PA, United States; Lisa Grandinetti, MD, University of Pittsburgh Medical Center, Pittsburgh, PA, United States
Introduction: Primary cutaneous gamma-delta T cell lymphoma (CGD-TCL) is a rare form of cutaneous T cell lymphoma (CTCL) associated with a poor prognosis and recently categorized with peripheral T cell lymphomas of the skin. Case presentation: A 65-year-old Caucasian female presented for evaluation of ‘‘nodules’’ on her lower extremities. In 2012, she developed tender, pruritic nodules in her right calf with additional lesions appearing over the next several months. On examination there were multiple erythematous subcutaneous nodules on her bilateral lower extremities, buttocks, and right upper arm. Staging: Multiple biopsies from lesions showed an atypical T cell lymphoid infiltrate with prominent granulomatous inflammation involving the deep dermis. Most lymphocytes expressed CD3 with a CD4:CD8 ratio of 1:1 with a subpopulation also positive for CD56, TCR gamma, and TIA-1. The interpretation was CGD-TCL with prominent granulomatous inflammation. Bone marrow and nodes showed no evidence of lymphoma. Initial PET/CT scan revealed abnormal uptake in the subcutaneous tissue of the trunk and extremities. ANA and anti-ds DNA were negative. Treatment: The patient initially used clobetasol ointment but progressed. Her lesions were controlled on oral bexarotene 225 mg daily and prednisone 20 mg daily, which was decreased to 10 mg over four months. In January 2014, hydroxychloroquine 200 mg BID was added. As she developed new lesions, confirmed with PET/CT imaging, her prednisone therapy was increased to 40 mg daily. Future treatment options include romidepsin, a CD3-diphtheria fusion protein; praletrexate, a folic acid inhibitor; total body electron beam radiation and allotransplant; or standard chemotherapy. Discussion: This case highlights a rare form of panniculitis-like cutaneous lymphoma, which represents \1% of all CTCL. Autoimmune diseases have been reported in 20% of patients with the alpha-beta phenotype. Our patient has the gamma-delta phenotype, which is associated with a very aggressive clinical course with a five year survival of \10%. Her course has been more indolent with partial response to bexarotene and prednisone. This case highlights new and experimental treatment options for peripheral T-cell lymphomas. Conclusion: Primary CGD-TCL is a rare form of peripheral T-cell lymphoma arising in the skin and may mimic inflammatory panniculitis. Prompt excisional biopsy and recognition is crucial in determining the disease course and treatment. Commercial support: None identified.
Cutaneous T-cell lymphoma (CTCL) is a type of non-Hodgkin lymphoma that involves proliferation of malignant CD4+ T-cells with possible involvement of other organs. CTCL and concurrent B-cell chronic lymphocytic leukemia (CLL) is rare, but has been previously reported in a handful of case reports. Simultaneous diagnosis of primary CTCL, CLL, and acute myeloid leukemia (AML) is a previously undocumented phenomenon that presents unique treatment challenges. Case report: A 77-year-old Caucasian male presented with a 10-week history of a severely pruritic, papular dermatitis on his face, arms, and chest. A punch biopsy revealed folliculotropic mycosis fungoides (MF). An excisional lymph node biopsy of palpable lymph nodes revealed a B-cell neoplasm most consistent with CLL, with no histologic support for a T-cell neoplasm. Further evaluation including a bone marrow biopsy revealed increased blasts and promonocytes of 24%, consistent with AML type M5. Patient declined treatment for CLL and AML. The main issue affecting his quality of life was severe skin pruritus. To select therapy for his MF and AML, the therapeutic choices included retinoids. However, patient was unable to tolerate even low dose bexarotene therapy due to recurrent severe neutropenia, necessitating G-CSF (filgrastim) injections. Extracorporeal photopheresis every 2 weeks brought some improvement in his pruritus. Ultimately, his AML progressed rapidly and the patient passed away. Conclusion: We present this patient diagnosed with consecutive triple primary hematologic malignancies, including CTCL, B-cell CLL, and AML, to highlight the difficulty in treating CTCL in patients with other hematologic malignancies. Bexarotene is a well-established treatment for CTCL; it is also believed to have antileukemic activity and to be well tolerated in nonM3 AML. Therefore, despite using intervention known to be safe in patients with CTCL or AML, it was a challenge to treat his CTCL with his concurrent CLL and AML due to recurring bone marrow suppression. Although multiple studies have investigated these interventions, none address its risks in patients with concurrent disease. The incidence of second malignancies in patients with CTCL is high. Case studies of CLL and other malignancies with CTCL have been reported. However, it is rare to have three hematologic malignancies in the same patient and they pose significant therapeutic challenges confounded by significant bone marrow suppression. Commercial support: None identified.
1660 Severe leonine facies in a case of folliculotropic mycosis fungoides Casey Wang, MD, University of Texas MD Anderson Cancer Center, Houston, TX, United States; Madeleine Duvic, MD, University of Texas MD Anderson Cancer Center, Houston, TX, United States; Bouthaina Dabaja, MD, University of Texas MD Anderson Cancer Center, Houston, TX, United States; Rakshandra Talpur, MD, University of Texas MD Anderson Cancer Center, Houston, TX, United States Introduction: Folliculotropic mycosis fungoides (FMF) is a rare variant of mycosis fungoides (MF) in which atypical T-cells infiltrate and destroy hair follicles and occasionally sweat glands (termed syringotropic). Unlike classic MF, FMF rarely exhibits epidermotropism. Morphologically, FMF is associated with acneiform lesions, pustules, cystic milia-like lesions, and follicular mucinosis. Leonine facies is an uncommon but potentially devastating development, resulting from nodular infiltration of the face. We present a patient with aggressive FMF with large cell transformation who rapidly developed severe leonine facies and was resistant to systemic chemotherapy. Case report: A 67-year-old African American male presented for treatment of severe facial swelling. The patient’s symptoms began 1 year ago with irritation of his chin. A skin biopsy was consistent with mycosis fungoides. He had progressive disease on topical medications, oral bexarotene, 5 cycles of R-CHOP, and 3 cycles of gemcitabine and carboplatin. 2 weeks prior to presentation, the patient developed progressive facial swelling that compromised his eyesight, hearing, and speech. The skin examination was significant for profound leonine facies, large confluent hyperpigmented plaques on his trunk, and scattered annular erosions with scale. A biopsy of his cheek showed a lymphocytic infiltrate of large cells in the dermis with folliculotropism, focal epidermotropism, and CD4:CD8 ratio [ 10. Peripheral blood flow cytometry showed absolute CD4+CD26e cell count of 2032 uL consistent with Sezary syndrome. FNA biopsy of an axillary node showed involvement by large cells. A bone marrow biopsy showed T-cell lymphoma comprising 20% of the marrow cellularity. While admitted, the patient was treated with 2500 cGy of local radiation to his face with improvement. However he developed exfoliation and dryness of his face that resulted in large bleeding fissures. Wound culture showed colonization with MRSA. During his treatment, he developed altered mental status and fevers. He ultimately succumbed to infection.
1944 The mantle cell masquerade Sphoorthi Jinna, MD, MS, University of Connecticut Health Center, Farmington, CT, United States; Jim Whalen, MD, University of Connecticut Health Center, Farmington, CT, United States Background: Mantle cell lymphoma (MCL) is a relatively rare type of non-Hodgkin lymphoma that commonly affects extranodal sites. Lesions typically occur in the gastrointestinal tract or bone marrow, with cutaneous manifestations being relatively uncommon. Clinical presentation can be diverse and is generally a result of metastasis. Given 90% of MCL patients currently present with stage III-IV disease, a high index of suspicion for unique presentations can result in earlier diagnosis. We report a case of an unusual cutaneous manifestation of MCL, namely as a deep facial subcutaneous nodule. Case description: A 76-year-old male with 3-6 month history of multiple painless enlarging subcutaneous nodules on his left cheek presented for evaluation. One nodule was excised for both diagnostic and therapeutic indications. Pathology revealed mantle cell lymphoma and the patient was promptly referred to oncology for further management.
Discussion: Folliculotropic mycosis fungoides is a therapeutic challenge as it is often resistant to standard therapy. The role of radiation in the treatment of leonine facies is unclear. Several studies report that FMF is associated with worse prognosis, comparable to that of classic tumor stage MF. Large cell transformation is also a poor prognostic factor and is associated with more advanced stages of MF.
Discussion: Given mantle cell lymphoma generally presents advanced stage and therefore with a poor prognosis, early detection is of paramount importance, especially in the wake of new systemic therapies. Case reports of cutaneous manifestations of MCL presenting as maculopapular rash or infiltrated plaques have been reported, but we report on the unusual presentation of MCL as a deep facial subcutaneous nodule.
Commercial support: None identified.
Commercial support: None identified.
MAY 2015
J AM ACAD DERMATOL
AB163