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Bullous Pyoderma Gangrenosum Associated with Hairy Cell Leukemia and Its Complete Response to Cladribine Therapy

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Abstract

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis seen in association with systemic disorders including hematologic malignancies. Hairy cell leukemia (HCL) however, is an unusual association of PG. We describe a 49-year old lady who presented to our hematology clinic with easy fatiguability and ulcerative skin lesions of 6 months duration. Examination revealed pallor and massive splenomegaly. Indurated, ulcerated lesion with undermined edges and necrotic base was observed on left thigh. Investigations revealed pancytopenia and bone marrow examination identified typical hairy cells. Flow cytometry of marrow aspirate was suggestive of classical HCL. BRAF V600E mutation was detected in peripheral blood by reverse transcriptase polymerase chain reaction. Skin biopsy revealed neutrophilic dermatosis and findings classical of bullous PG. Cladribine therapy (0.09 mg/kg/day by continuous intravenous infusion for 7 days) led to remission of both HCL and PG after a duration of 4 weeks. Cladribine monotherapy in a case of PG with HCL may avoid the additional immunosuppresion risk imposed by treating PG separately with corticosteroids. Immunosuppressive role of cladribine might be helpful in treating PG concurrent with HCL.

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Acknowledgments

Ankur Jain: wrote the draft and managed the case, Deepesh Lad: supervised the case management, Gaurav Prakash and Alka Khadwal: supervised in diagnosis of the case, Pankaj Malhotra and Subhash Varma: involved in therapy related decisions, Amanjit Bal: reviewed the skin biopsy, Nabhajit Mallik and Narendar Kumar: reviewed the bone marrow biopsy of the patient.

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Authors and Affiliations

  1. Department of Internal Medicine and Hematology, 4th Floor, Nehru Hospital, PGIMER, Chandigarh, India

    Ankur Jain, Deepesh Lad, Gaurav Prakash, Alka Khadwal, Pankaj Malhotra & Subhash Varma

  2. Department of Histopathology, PGIMER, Chandigarh, 160012, India

    Amanjit Bal

  3. Department of Hematopathology, 5th Floor, Research Block A, PGIMER, Chandigarh, India

    Nabhajit Mallik & Narendar Kumar

Authors
  1. Ankur Jain
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  2. Deepesh Lad
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  3. Gaurav Prakash
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  4. Alka Khadwal
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  5. Pankaj Malhotra
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  6. Amanjit Bal
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  7. Nabhajit Mallik
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  8. Narendar Kumar
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  9. Subhash Varma
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Corresponding author

Correspondence to Pankaj Malhotra.

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The authors state that written consent was obtained from the patient prior to publication of any material relating to him.

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The authors declare that the article follows the required ethical standards as per Helsinki’s declaration.

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Jain, A., Lad, D., Prakash, G. et al. Bullous Pyoderma Gangrenosum Associated with Hairy Cell Leukemia and Its Complete Response to Cladribine Therapy. Indian J Hematol Blood Transfus 33, 273–275 (2017). https://doi.org/10.1007/s12288-016-0713-8

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  • DOI: https://doi.org/10.1007/s12288-016-0713-8

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