The simultaneous presentation of peripheral T-cell lymphoma and hairy cell leukemia

Abstract

A patient who presented simultaneously with B hairy cell leukemia (HCL) and peripheral T-cell lymphoma (ITL) is described. The diagnoses of the two neoplasms were made by standard morphologic and cytochemical study and confirmed immunologically. There was no evidence of overlap in markers to suggest that they arose from a single clone of malignant cells. It is suggested that the simultaneous occurrence of the two neoplasms in the same patient reflects an underlying predisposition to the development of neoplasia in HCL.Cancer 60:1537-1544, 1987.AIRY CELL LEUKEMIA (HCL) is a chronic lympho-H proliferative disorder characterized clinically by splenomegaly, pancytopenia and the presence of abnormal cells which usually show marked cytoplasmic projections, the so-called "hairy cells," and histologically confirmed by specific spleen and bone marrow appearances. The majority of cases of HCL have been shown to be of B-cell origin' although rare but well documented cases of HCL with hybrid T/B phenotypes or T-cell phenotypes have been Although data from a large series of cases of HCL is lacking, there is evidence that second neoplasms are common in HCL.' Second lymphoid malignancies reported with HCL include large cell lymphoma9.'' multiple myeloma,"-'3 and SCzary ~y n d r o m e ,~ and it has been tempting to postulate that in these cases both neoplasms arise from the same clone. Zucker-Franklin et a/.' described a case of T-HCL and Skzary syndrome where the hairy cells and SCzary cells had the same immunological phenotype and appeared to arise from the same clone. However, in two of three cases of multiple myeloma and HCL" it was found that both the heavy and light chain classes of the paraprotein were different from the immunoglobulins on the hairy cells suggesting that they were in fact from separate clones. Accepted for publication April 6, 1987.We have recently studied a patient who presented simultaneously with peripheral T-cell lymphoma (PTL)I4 and B-HCL. No evidence of overlap was found in tumor markers and the HCL and PTL appeared to represent the co-existence of two diseases either randomly or more likely as a result of an underlying predisposition to neoplasia in HCL. Case ReportA 65-year-old school teacher presented in May 1983 with a raised nontender 1.5 cm diameter lesion on her chin which had developed over 2 months. This was removed and reported histologically to be an undifferentiated carcinoma. In July 1983 a left parietal scalp lesion developed and after biopsy and review of the earlier specimen, a diagnosis of large cell lymphoma was made. She had no systemic complaints, no previous illness and no exposure to radiation. There was no hepatomegaly splenomegaly or lymphadenopathy.Hematologic investigations showed a hemoglobin (Hb) of 1 1.7 gm/dl, platelet count 97 X lo9 /I and leukocyte count 2.2 X 109/1: polymorphs 3 I%, lymphocytes 50%, eosinophils 4%, monocytes 1 %, hairy cells 8%, and atypical lymphocytes with convoluted nuclei 6% (Figs. 1A and IB). Occasional tartrate resistant acid phosphatase (TRAP...

Second Cancer Risk in Hairy Cell Leukemia

“…In summary, the possible mechanisms to explain the development of second or even tertiary cancers in patients treated for HCL include: presence of cell-mediated immune deficits 4 ; the expression of proto-oncogenes by such patients leading to the transformation and development of second tumors expressing the same proto-oncogenes 7 ; the immunological deficits produced by the treatment modalities including splenectomy 8 ; direct oncogenic defect of agents like IFN-α 3 ; old age further contributing to immune dysfunction 9 ; possibility of genetic components; shared etiological factors and environmental factors; and last but not the least, the observed increase in the incidence of second neoplasm in this group of patients who are immunocompromised because of HCL and thus prone to develop second tumors. If this is the case, then the frequency of second neoplasms in patients with HCL may be even greater in the future with continued improvements in therapy.…”

“…Previously, the high incidence of second neoplasms in patients with HCL had been attributed to the chemotherapeutic agents causing a worsening of immunological defects which may allow clonal mutation and hence development of second cancer. 4 There is also a possibility of IFN-α therapy having some direct oncogenic defect, evidenced by a significant decrease in CD4 helper cells in one of our patients. 3 An incidence of 19% has been reported in association with IFN.…”

Coexistent hairy cell leukaemia and hepatosplenic t-cell lymphoma: a case report

“…Among malignancies with synchronous occurrence, the most frequent is the occurrence with other haematopoietic malignancies [14-21], B-cell lymphomas in the first place, and very rarely also with T-cell neoplasms. Contemporary literature provides only rare case reports describing the coexistence of HCL with LGL leukaemia [20] and simultaneous presentation of HCL and peripheral T-cell lymphoma [19]. …”

Synchronous Occurrence of Neuroendocrine Colon Carcinoma and Hairy Cell Leukemia

“…The exact etiology of second malignancies in patients with HCL has not been clarified. Possible mechanisms include immunological deficits associated with all types and combinations of treatments, including splenectomy alone [5], defective cell-mediated immunity in patients with HCL [14], whereas the expression of protooncogenes by HCL may lead to transformation and development of a second tumor expressing the same protooncogenes [15].…”