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Serum IgG4 is typically measured for Immunoglobulin G4-related Disease (IgG4-RD), a fibroinflammatory condition associated with polyclonal increase in serum IgG4. Yet, increased IgG4 may still be monoclonal, and little is known about IgG4 POEMS syndrome. We present a case of 40-year-old male with a mass lesion in the left sacral ala. The mass was composed of non-neoplastic fibrous tissue and dense infiltrate of mature plasmacytes with dense eosinophilic cytoplasm and eccentrically placed nuclei that express monoclonal Lambda free light chains and show diffuse positivity for IgG and IgG4. We discuss clinical manifestations and challenges encountered in the diagnosis and treatment of this rare coexistence.

Introduction: Chronic lymphocytic leukemia (CLL) that arises by malignant transformation of mature B cell is the most common leukemia in elderly. CLL is characterized by accumulation of CD5(+)CD19(+) cells in the peripheral blood and lymphoid organs. While CD8(+) T lymphocytes response intracellular pathogen and tumor, CD4(+) T lymphocytes regulate immune response. T follicular (Tfol) cells stimulate affinity maturation and class-switch recombination in B lymphocytes due to cytokine and surface molecules. In this study, we aimed to investigate CD4(+)T, CD8(+)T and CD3(+)CD4(+)CXCR5(+) follicular T (Tfol) cells in the peripheral blood of the CLL patients compared to healthy controls.

To the Editor Hairy cell leukemia (HCL) is a mature B-cell lymphoid neoplasm characterized by infiltration of bone marrow and splenic red pulp by small mature cells with oval nuclei and abundant cytoplasm with hairy projections [1]. HCL is a rare disease, constituting 2% of lymphoid leukemias. At initial diagnosis, patients are mostly middle-aged to elderly with a median age of 52 years with a male to female ratio of 5:1 [1]. Chronic myeloproliferative neoplasms (MPNs) represent a group of heterogeneous diseases of clonal origin characterized by abnormal proliferation of one or several myeloid lineages. Polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) represent the three classical Philadelphia (Ph)-negative MPNs. Patients with ET and PV have life expectancies comparable with that of age-matched healthy individuals [2]. A population-based study including 3104 HCL patients demonstrated that patients with HCL are at increased risk of Hodgkin's lymphoma (HL), non-Hodgkin's lymphoma (NHL) and solid cancers including thyroid cancer [1]. That study suggested that future studies are needed to address the role of immunologic impairment inherent to HCL, treatment modalities and other factors in the increased risk of second malignancies [1]. In contrast, in another long-term follow-up study including 241 HCL patients, subsequent malignancies did not appear to increase after pentostatin treatment [3]. Furthermore, the study by Federico et al. did not support the contention that HCL poses an increased risk for additional second malignancies, although the incidence of lymphoid neoplasms was significantly higher than expected [4]. The association of a Ph-negative MPN and lymphoproliferative neoplasm (LPN) in the same patient is a relatively uncommon event and has been reported mostly as case reports. Recently, in a study including the largest number of Ph-negative MPN and LPN patients, the incidence of concurrent Ph-negative MPN and LPN was reported as 0.3% [5]. LPN was diagnosed first in 47.1%, second in 44.1% and concurrently in 8.8% of patients [5]. The most common types of LPN were NHL and chronic lymphocytic leukemia (CLL) followed by HL and multiple myeloma (MM) [5]. PMF (41%), PV (24%) and ET (18%) were the most common MPNs, followed by MPN unclassifiable (MPN, U) and hypereosinophilic syndrome (HES) [5]. To our knowledge, there are only few reports describing a PV patient who subsequently develops HCL [6, 7]. Moreover, to date, only three reports have described a diagnosis of HCL variant developing in a background of PV [8–10]. We now report the first case of PV developed 3 years after the diagnosis of HCL. A 58-year-old man presented 3 years earlier with fatigue, and his complete blood count showed the following: leukocyte 1400/mm3 with 42.2% segmented neutrophils, 40% lymphocytes and 15.3% monocytes, hemoglobin 8 g/dl, MCV 107 fL and platelets 19 000/mm3. His past medical history was significant for diabetes mellitus, hypertension and dyslipidemia. On physical examination, there was splenomegaly measuring 6 cm below the costal margin. Peripheral smear showed leukopenia with atypical lymphocytes displaying cytoplasmic projections (Fig. 1). Bone marrow biopsy demonstrated diffuse neoplastic infiltration consisting of cells with round, oval, regular nuclei and medium-sized, clear cytoplasm. Neoplastic cells expressed CD20, tartrate-resistant acid phosphatase (TRAP), LCA, CD79 alpha, CD11c, CD68 and annexin A1. Flow cytometry of bone marrow showed coexpression of B-cell marker CD19 and CD11c, CD25, CD103 and FMC7. Findings were consistent with HCL. Chemotherapy with the purine analog cladribine (2-chlorodeoxyadenosine) at a dose of 0.1 mg/kg per day for 7 days as continuous infusion was administered. Three months after the chemotherapy, complete remission was obtained with disappearance of hairy cells in bone marrow and splenomegaly. He remained disease free at regular follow-up visits. Approximately 3 years after the diagnosis of HCL, he was examined for persistent headache. His complete blood count showed a hemoglobin level of 18.6 g/l, hematocrit of 55% and no other abnormalities. Examination of his arterial blood gases found no hypoxemia; erythropoietin level was 2.3 U/l (2.6–18.5), and lactate dehydrogenase (LDH) value was 520 U/l (normal value

We describe an unusual case of hairy cell leukemia (HCL) in a 55-year-old male presenting with isolated skeletal disease as the initial manifestation without abnormal peripheral blood counts, bone marrow involvement, or splenomegaly. To the best of our knowledge, there have been only two previous reports of a similar case. The patient presented with pain in the right femur. Anteroposterior radiographs of both femurs revealed mixed lytic-sclerotic lesions. PET scan showed multiple metastatic lesions on axial skeleton, pelvis, and both femurs. Histopathological examination of the bone biopsy revealed an infiltrate of HCL. Localized radiation therapy to both proximal femurs and subsequently 4 weeks later, a 7-day course of 0.1 mg/kg/day cladribine provided complete remission with relief of symptoms and resolution of bone lesions. We addressed the manifestations and management of HCL patients with skeletal involvement.