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Hairy cell leukemia in kidney transplantation: lesson from a rare disorder.

Vinante F, Tomei P, Zaza G, Zamò A, Lupo A - Exp Hematol Oncol (2013)

Bottom Line: We report here on the diagnosis and successful treatment of a case of hairy cell leukemia (HCL) that arose 15 years after kidney transplantation in a 51-year-old patient.Recently, however, the oncogenic mutation V600E of the BRAF protein kinase has been found to be a hallmark of HCL, and calcineurin inhibitors have been shown to interfere with signaling downstream of V600E BRAF early on by counteracting senescence-associated mechanisms that protect against the oncogenic potential of the mutated kinase.This mechanism might also be involved in other neoplasias bearing the same or similar mutations, such as melanoma and non-melanoma skin cancer.

View Article: PubMed Central - HTML - PubMed

Affiliation: Section of Hematology, Department of Medicine, University of Verona, Verona, Italy.

ABSTRACT
We report here on the diagnosis and successful treatment of a case of hairy cell leukemia (HCL) that arose 15 years after kidney transplantation in a 51-year-old patient. As soon as the diagnosis was made, HCL was treated with 2-CDA, obtaining complete hematological remission. Immunosuppression with the calcineurin inhibitor cyclosporin was maintained, and the graft was preserved. In kidney transplant recipients supported with immunosuppressive drugs, post-transplant lymphoproliferative diseases (PTLDs) are frequent and typically related to immunosuppression via a loss of control of infectious/EBV-related proliferative stimuli. To date, HCL has not been considered among PTLDs. Recently, however, the oncogenic mutation V600E of the BRAF protein kinase has been found to be a hallmark of HCL, and calcineurin inhibitors have been shown to interfere with signaling downstream of V600E BRAF early on by counteracting senescence-associated mechanisms that protect against the oncogenic potential of the mutated kinase. Such a biochemical link between the oncogene-dependent signaling and calcineurin inhibitor activities suggests that HCL in transplanted patients might be a peculiar type of PTLD based on the presence of a specific mutation. This mechanism might also be involved in other neoplasias bearing the same or similar mutations, such as melanoma and non-melanoma skin cancer.

No MeSH data available.


Related in: MedlinePlus

Bone marrow biopsy. Infiltrate of mature lymphoid cells (hematoxylin & eosin, H/E), which stained for CD20 (B cell marker), DBA44 and V600E BRAF (HCL markers). Histology and immunohistology were performed according to standard diagnostic procedures.
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Figure 1: Bone marrow biopsy. Infiltrate of mature lymphoid cells (hematoxylin & eosin, H/E), which stained for CD20 (B cell marker), DBA44 and V600E BRAF (HCL markers). Histology and immunohistology were performed according to standard diagnostic procedures.

Mentions: A physical examination, renal allograft function, abdominal ultrasonography, thoracic Rx and rheumatologic and infectious disease screening were unremarkable. Because the pancytopenia was persistent, peripheral blood and bone marrow examinations were performed. A peripheral blood smear and a flow cytometric study of circulating mononuclear cells failed to show diagnostic cells. Bone marrow could not be aspirated. Biopsy showed severely hypocellular marrow with few infiltrating B cells, which suggested lymphoproliferative disease. A contralateral biopsy showed a pattern of marrow infiltration by mature B lymphocytes that was diagnostic of HCL (Figure 1).


Hairy cell leukemia in kidney transplantation: lesson from a rare disorder.

Vinante F, Tomei P, Zaza G, Zamò A, Lupo A - Exp Hematol Oncol (2013)

Bone marrow biopsy. Infiltrate of mature lymphoid cells (hematoxylin & eosin, H/E), which stained for CD20 (B cell marker), DBA44 and V600E BRAF (HCL markers). Histology and immunohistology were performed according to standard diagnostic procedures.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3750510&req=5

Figure 1: Bone marrow biopsy. Infiltrate of mature lymphoid cells (hematoxylin & eosin, H/E), which stained for CD20 (B cell marker), DBA44 and V600E BRAF (HCL markers). Histology and immunohistology were performed according to standard diagnostic procedures.
Mentions: A physical examination, renal allograft function, abdominal ultrasonography, thoracic Rx and rheumatologic and infectious disease screening were unremarkable. Because the pancytopenia was persistent, peripheral blood and bone marrow examinations were performed. A peripheral blood smear and a flow cytometric study of circulating mononuclear cells failed to show diagnostic cells. Bone marrow could not be aspirated. Biopsy showed severely hypocellular marrow with few infiltrating B cells, which suggested lymphoproliferative disease. A contralateral biopsy showed a pattern of marrow infiltration by mature B lymphocytes that was diagnostic of HCL (Figure 1).

Bottom Line: We report here on the diagnosis and successful treatment of a case of hairy cell leukemia (HCL) that arose 15 years after kidney transplantation in a 51-year-old patient.Recently, however, the oncogenic mutation V600E of the BRAF protein kinase has been found to be a hallmark of HCL, and calcineurin inhibitors have been shown to interfere with signaling downstream of V600E BRAF early on by counteracting senescence-associated mechanisms that protect against the oncogenic potential of the mutated kinase.This mechanism might also be involved in other neoplasias bearing the same or similar mutations, such as melanoma and non-melanoma skin cancer.

View Article: PubMed Central - HTML - PubMed

Affiliation: Section of Hematology, Department of Medicine, University of Verona, Verona, Italy.

ABSTRACT
We report here on the diagnosis and successful treatment of a case of hairy cell leukemia (HCL) that arose 15 years after kidney transplantation in a 51-year-old patient. As soon as the diagnosis was made, HCL was treated with 2-CDA, obtaining complete hematological remission. Immunosuppression with the calcineurin inhibitor cyclosporin was maintained, and the graft was preserved. In kidney transplant recipients supported with immunosuppressive drugs, post-transplant lymphoproliferative diseases (PTLDs) are frequent and typically related to immunosuppression via a loss of control of infectious/EBV-related proliferative stimuli. To date, HCL has not been considered among PTLDs. Recently, however, the oncogenic mutation V600E of the BRAF protein kinase has been found to be a hallmark of HCL, and calcineurin inhibitors have been shown to interfere with signaling downstream of V600E BRAF early on by counteracting senescence-associated mechanisms that protect against the oncogenic potential of the mutated kinase. Such a biochemical link between the oncogene-dependent signaling and calcineurin inhibitor activities suggests that HCL in transplanted patients might be a peculiar type of PTLD based on the presence of a specific mutation. This mechanism might also be involved in other neoplasias bearing the same or similar mutations, such as melanoma and non-melanoma skin cancer.

No MeSH data available.


Related in: MedlinePlus