LETTER TO THE EDITOR
Hairy cell leukemia presenting with progressive pericarditis and pleuritis
Received: 23 April 2018 / Accepted: 21 May 2018
The Author(s) 2018
Hairy cell leukemia (HCL) has been recognized as an indolent
B cell leukemia characterized by splenomegaly, pancytopenia,
and neoplastic cells morphologically with irregular cytoplas-
mic hair-like projections on smears . However, body fluid
retention, such as ascites and pleural effusion, are extremely
rare in HCL [2–4].
A 43-year-old Japanese female was referred to our hospital
because of a 2-month history of fever and general fatigue. She
had suffered from appetite loss and abdominal fullness for
2 years before admission. Physical examination revealed ane-
mia and palpable spleen. Lymph nodes were not palpable.
Blood examination showed leukocyte count of 3.5 × 10
with 81% atypical lymphocytes. Soluble IL-2 receptor was
43,974 U/mL. The atypical lymphocytes were medium-sized
with hair-like cytoplasmic projections and were positive for
CD19, CD20, CD22, CD11c, CD25, FMC7, and CD103.
Images obtained from phase contrast microscopy and trans-
mission electron microscopy (Fig. 1a) also showed hairy cy-
toplasmic projections along the cellular border. The fried-egg
appearance with diffuse infiltration of atypical cells was ob-
served on bone marrow biopsy.
Inflammatory conditions have continued with high fever
and serum C-reactive protein levels around 20 mg/dL. Chest
X-ray showed pleural effusion and cardiomegaly (Fig. 1b).
Echocardiography exhibited pericardial effusion (Fig. 1c).
Since dyspnea and hypotension were observed on day 8 after
admission, the pericardial and pleural effusions were drained.
The effusions were exudative with cell infiltration consisting
of atypical lymphocytes (Fig. 1d) and neutrophils. Atypical
lymphocytes in the effusions were also positive for CD19,
CD20, CD22, CD11c, CD25, FMC7, and CD103. Extensive
laboratory testing for autoimmune diseases, bacteria, virus,
fungus, and tubercle bacillus were all negative.
IL-6 production has been demonstrated in HCL leukemic
cells [5, 6]. In our reverse transcription-polymerase chain re-
action analysis, her effusion HCL cells but not her peripheral
blood HCL cells showed significant expression of IL-6
mRNA. A remarkably high level of IL-6 (2900 pg/mL) was
also observed in the pericardial effusion, compared with that
in serum (5.42 pg/mL). G-CSF levels were elevated in both
serum (318 pg/mL) and the effusion (2530 pg/mL). Neither
TNFα nor GM-CSF was elevated. Methylprednisolone ad-
ministration improved her inflammatory conditions such as
fever. The pericardial and pleural effusions were significantly
decreased after the first cycle of pentostatin (5 mg/m
However, after the first cycle, pentostatin was discontinued
due to skin rash in her trunk and extremities, which were
suspected to be caused by pentostatin. Therefore, she was
treated with cladribine (0.09 mg/kg for 7 days), which
completely resolved both pleuritis and pericarditis. There has
been no recurrence of pleuropericarditis as well as HCL for
To our knowledge, this is the first report of HCL presenting
with progressive pericarditis. Early recognition and interven-
tion prolong survival in patients with malignant pericardial
effusion, especially in those with chemotherapy-sensitive ma-
lignancies such as lymphoma. In our case, an initial relief of
the symptoms was obtained by effusion drainage. Further im-
provement was achieved with systemic chemotherapy.
Elevated concentrations of IL-6 have been shown in pericar-
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
was detected only in the effusion HCL cells, suggesting that
effusion IL-6 might be involved in the fluid retention and
systemic inflammatory responses.
Because the patient has died of breast cancer brain metas-
tasis and her kin are not traceable, the ethics committee of our
institute has approved publication of this report.
* Junichi Tsukada
Hematology, University of Occupational and Environmental Health,
1-1 Iseigaoka, Yahatanishi, Kitakyushu 807-8556, Japan
Palliative Care Center, University of Occupational and
Environmental Health, Kitakyushu, Japan
Annals of Hematology