Annals of Hematology https://doi.org/10.1007/s00277-018-3378-6 LETTER TO THE EDITOR 1,2 1 1 Atsushi Iwashige & Makoto Hirosawa & Junichi Tsukada Received: 23 April 2018 / Accepted: 21 May 2018 The Author(s) 2018 Dear Editor, CD20, CD22, CD11c, CD25, FMC7, and CD103. Extensive Hairy cell leukemia (HCL) has been recognized as an indolent laboratory testing for autoimmune diseases, bacteria, virus, B cell leukemia characterized by splenomegaly, pancytopenia, fungus, and tubercle bacillus were all negative. and neoplastic cells morphologically with irregular cytoplas- IL-6 production has been demonstrated in HCL leukemic mic hair-like projections on smears . However, body fluid cells [5, 6]. In our reverse transcription-polymerase chain re- retention, such as ascites and pleural effusion, are extremely action analysis, her effusion HCL cells but not her peripheral rare in HCL [2–4]. blood HCL cells showed significant expression of IL-6 A 43-year-old Japanese female was referred to our hospital mRNA. A remarkably high level of IL-6 (2900 pg/mL) was because of a 2-month history of fever and general fatigue. She also observed in the pericardial effusion, compared with that had suffered from appetite loss and abdominal fullness for in serum (5.42 pg/mL). G-CSF levels were elevated in both 2 years before admission. Physical examination revealed ane- serum (318 pg/mL) and the effusion (2530 pg/mL). Neither mia and palpable spleen. Lymph nodes were not palpable. TNFα nor GM-CSF was elevated. Methylprednisolone ad- Blood examination showed leukocyte count of 3.5 × 10 /L ministration improved her inflammatory conditions such as with 81% atypical lymphocytes. Soluble IL-2 receptor was fever. The pericardial and pleural effusions were significantly 43,974 U/mL. The atypical lymphocytes were medium-sized decreased after the first cycle of pentostatin (5 mg/m ). with hair-like cytoplasmic projections and were positive for However, after the first cycle, pentostatin was discontinued CD19, CD20, CD22, CD11c, CD25, FMC7, and CD103. due to skin rash in her trunk and extremities, which were Images obtained from phase contrast microscopy and trans- suspected to be caused by pentostatin. Therefore, she was mission electron microscopy (Fig. 1a) also showed hairy cy- treated with cladribine (0.09 mg/kg for 7 days), which toplasmic projections along the cellular border. The fried-egg completely resolved both pleuritis and pericarditis. There has appearance with diffuse infiltration of atypical cells was ob- been no recurrence of pleuropericarditis as well as HCL for served on bone marrow biopsy. 5years. Inflammatory conditions have continued with high fever To our knowledge, this is the first report of HCL presenting and serum C-reactive protein levels around 20 mg/dL. Chest with progressive pericarditis. Early recognition and interven- X-ray showed pleural effusion and cardiomegaly (Fig. 1b). tion prolong survival in patients with malignant pericardial Echocardiography exhibited pericardial effusion (Fig. 1c). effusion, especially in those with chemotherapy-sensitive ma- Since dyspnea and hypotension were observed on day 8 after lignancies such as lymphoma. In our case, an initial relief of admission, the pericardial and pleural effusions were drained. the symptoms was obtained by effusion drainage. Further im- The effusions were exudative with cell infiltration consisting provement was achieved with systemic chemotherapy. of atypical lymphocytes (Fig. 1d) and neutrophils. Atypical Elevated concentrations of IL-6 have been shown in pericar- lymphocytes in the effusions were also positive for CD19, dial effusion associated with hematological malignancies [7–9]. In our case, an extremely high IL-6 concentration was observed in the pericardial effusion. IL-6 mRNA expression * Junichi Tsukada was detected only in the effusion HCL cells, suggesting that firstname.lastname@example.org effusion IL-6 might be involved in the fluid retention and systemic inflammatory responses. Hematology, University of Occupational and Environmental Health, Because the patient has died of breast cancer brain metas- 1-1 Iseigaoka, Yahatanishi, Kitakyushu 807-8556, Japan tasis and her kin are not traceable, the ethics committee of our Palliative Care Center, University of Occupational and institute has approved publication of this report. Environmental Health, Kitakyushu, Japan Ann Hematol Fig. 1 Transmission electron AB microscopy demonstrated hairy cytoplasmic projections along the cellular border of peripheral blood atypical lymphoid cells (a). Chest X-ray showed pleural effusion and cardiomegaly (b). Echocardiography examination exhibited pericardial effusion (c). Atypical lymphocytes with hairy cytoplasmic projections were ob- served in the pericardial effusion CD (d; Wright-Giemsa staining × 400) Pericardial effusion 4. Tadmor T, Polliack A (2015) Hairy cell leukemia: uncommon clin- Compliance with ethical standards ical features, unusual sites of involvement and some rare associa- tions. Best Pract Res Clin Haematol 28(4):193–199. https://doi.org/ Conflict of interest 10.1016/j.beha.2015.10.020 The authors declare that they have no conflict of interest. 5. Barut B, Chauhan D, Uchiyama H, Anderson KC (1993) Interleukin- 6 functions as an intracellular growth factor in hairy cell leukemia in Open Access This article is distributed under the terms of the Creative vitro. J Clin Invest 92(5):2346–2352 Commons Attribution 4.0 International License (http:// 6. Heslop HE, Bianchi AC, Cordingley FT, Turner M, Chandima W, De creativecommons.org/licenses/by/4.0/), which permits unrestricted use, Mel CP, Hoffbrand AV, Brenner MK (1990) Effects of interferon distribution, and reproduction in any medium, provided you give appro- alpha on autocrine growth factor loops in B lymphoproliferative priate credit to the original author(s) and the source, provide a link to the disorders. J Exp Med 172(6):1729–1734 Creative Commons license, and indicate if changes were made. 7. Takahashi R, Ashihara E, Hirata T, Okawa K, Oku N, Goto H, Inaba T, Fujita N, Shimazaki C, Nakagawa M (1994) Aggressive myeloma with subcutaneous tumor and pericardial involvement. Rinsho Ketsueki 35(3):291–295 References 8. Pankuweit S, Wadlich A, Meyer E, Portig I, Hufnagel G, Maisch B (2000) Cytokine activation in pericardial fluids in different forms of pericarditis. Herz 25(8):748–754 1. Bouroncle BA, Wiseman BK, Doan CA (1958) Leukemic 9. Ristic AD, Pankuweit S, Maksimovic R, Moosdorf R, Maisch B reticuloendotheliosis. Blood 13(7):609–630 (2013) Pericardial cytokines in neoplastic, autoreactive, and viral 2. Bouroncle BA (1979) Leukemic reticuloendotheliosis (hairy cell leu- pericarditis. Heart Fail Rev 18(3):345–353. https://doi.org/10.1007/ kemia). Blood 53(3):412–436 s10741-012-9334-y 3. Bouroncle BA (1987) Unusual presentations and complications of hairy cell leukemia. Leukemia 1(4):288–293
Annals of Hematology – Springer Journals
Published: May 30, 2018
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