Dissecting folliculitis (dissecting cellulitis) of the scalp: a 66-patient case series and proposal of classification.

BACKGROUND AND OBJECTIVES: Dissecting folliculitis (DF) or dissecting cellulitis of the scalp is regarded as a rare disease with disfiguring scarring alopecia. This study aimed to analyze the features of DF and to propose a classification to define its severity. PATIENTS AND METHODS: A hospital-based retrospective study was conducted. Patients with a histopathological diagnosis or clinical features leading to diagnosis of DF were included and classified into three stages. RESULTS: Among the 66 patients recruited (63 men / 3 women, mean age 24.9 years), multiple interconnected alopecic nodules involving the vertex scalp were the main feature. Histopathology showed an extensive inflamed granulation abscess forming a dissection plane in the lower dermis/subcutis in the acute stage. Lymphocytic infiltration was predominant in seven of 21 histology specimens. Overweight and obesity were noted in 29 of 45 patients examined. No association with smoking was found. There was comorbidity with acne conglobata in 15 of 66 patients, two of whom had acne inversa. Longer disease duration and greater number of nodules were associated with higher severity of DF (p < 0.05). A complete remission rate of 25 % was achieved by any treatment, and a rate of 37.5 % was achieved with oral isotretinoin alone. CONCLUSIONS: DF is not uncommon in Taiwan. An association with obesity needs to be clarified.

Lymphoma-like monoclonal B cell lymphocytosis in a patient population: biology, natural evolution, and differences from CLL-like clones.

High-count monoclonal B cell lymphocytosis (MBL) with a chronic lymphocytic leukemia (CLL) phenotype is a well-known entity, featuring 1-4% annual risk of progression towards CLL requiring treatment. Lymphoma-like MBL (L-MBL), on the other hand, remains poorly defined and data regarding outcome are lacking. We retrospectively evaluated 33 L-MBL cases within our hospital population and compared them to 95 subjects with CLL-like MBL (C-MBL). Diagnoses of L-MBL were based on asymptomatic B cell clones with Matutes score < 3, B cells < 5.0 × 103/µl, and negative computerized tomography scans. We found that median B cell counts were considerably lower compared to C-MBL (0.6 vs 2.3 × 103/µl) and remained stable over time. Based on immunophenotyping and immunogenetic profiling, most L-MBL clones did not correspond to known lymphoma entities. A strikingly high occurrence of paraproteinemia (48%), hypogammaglobulinemia (45%), and biclonality (21%) was seen; these incidences being significantly higher than in C-MBL (17, 21, and 5%, respectively). Unrelated monoclonal gammopathy of undetermined significance was a frequent feature, as the light chain type of 5/12 paraproteins detected was different from the clonal surface immunoglobulin. After 46-month median follow-up, 2/24 patients (8%) had progressed towards indolent lymphoma requiring no treatment. In contrast, 41% of C-MBL cases evolved to CLL and 17% required treatment. We conclude that clinical L-MBL is characterized by pronounced immune dysregulation and very slow or absent progression, clearly separating it from its CLL-like counterpart.

Clonal B-cell lymphocytosis of marginal zone origin.

Monoclonal B cell Lymphocytosis (MBL) is the term used to characterize individuals presenting with lymphocytosis in the absence of lymphadenopathy, organomegaly or any other features suggestive of an active disease. Based on the immunophenotypic findings, MBL cases are sub-categorized into chronic lymphocytic leukemia (CLL)-like, atypical CLL and non-CLL MBL. The latter corresponds to cases with immunophenotypic features suggestive of post germinal center derivation and still represents a diagnostic conundrum. Recent studies are starting to shed light on the true biological nature and clinical significance of this entity and have led to the introduction of the novel term clonal B lymphocytosis of marginal-zone origin (CBL-MZ); as well as the acknowledgement of CBL-MZ in the latest (2016) update of the WHO classification for lymphoid malignancies. Here we provide an overview of relevant research concerning non-CLL MBL and discuss clinico-biological implications and considerations.

Prospective study of prognostic factors in asymptomatic patients with B-cell chronic lymphocytic leukemia-like lymphocytosis: the cut-off of 11 × 10(9)/L monoclonal lymphocytes better identifies subgroups with different outcomes.

The arbitrary threshold of 5 × 10(9)/L chronic lymphocytic leukemia (CLL)-like lymphocytes differentiates monoclonal B lymphocytosis (MBL) from CLL. There are no prospective studies that search for the optimal cut-off of monoclonal lymphocytes able to predict outcome and simultaneously analyze the prognostic value of classic, immunophenotypic, and cytogenetic variables in patients with asymptomatic clonal CLL lymphocytosis (ACL), which includes MBL plus Rai 0 CLL patients. From 2003 to 2010, 231 ACL patients were enrolled in this study. Patients with 11q deletion and atypical lymphocyte morphology at diagnosis had shorter progression-free survival (PFS) (p = 0.007 and p = 0.015, respectively) and treatment-free survival (TFS) (p = 0.009 and p = 0.017, respectively). Elevated beta-2 microglobulin (B2M) also correlated with worse TFS (p = 0.002). The optimal threshold of monoclonal lymphocytes independently correlated with survival was 11 × 10(9)/L (p = 0.000 for PFS and p = 0.016 for TFS). As conclusion, monoclonal lymphocytosis higher than 11 × 10(9)/L better identifies two subgroups of patients with different outcomes than the standard cut-off value of 5 × 10(9)/L. Atypical lymphocyte morphology, 11q deletion and elevated B2M had a negative impact on the survival in ACL patients.

Clonal B-cell lymphocytosis exhibiting immunophenotypic features consistent with a marginal-zone origin: is this a distinct entity?

The biological and clinical significance of a clonal B-cell lymphocytosis with an immunophenotype consistent with marginal-zone origin (CBL-MZ) is poorly understood. We retrospectively evaluated 102 such cases with no clinical evidence to suggest a concurrent MZ lymphoma. Immunophenotyping revealed a clonal B-cell population with Matutes score ≤2 in all cases; 19/102 were weakly CD5 positive and all 35 cases tested expressed CD49d. Bone marrow biopsy exhibited mostly mixed patterns of small B-lymphocytic infiltration. A total of 48/66 (72.7%) cases had an abnormal karyotype. Immunogenetics revealed overusage of the IGHV4-34 gene and somatic hypermutation in 71/79 (89.8%) IGHV-IGHD-IGHJ gene rearrangements. With a median follow-up of 5 years, 85 cases remain stable (group A), whereas 17 cases (group B) progressed, of whom 15 developed splenomegaly. The clonal B-cell count, degree of marrow infiltration, immunophenotypic, or immunogenetic findings at diagnosis did not distinguish between the 2 groups. However, deletions of chromosome 7q were confined to group A and complex karyotypes were more frequent in group B. Although CBL-MZ may antedate SMZL/SLLU, most cases remain stable over time. These cases, not readily classifiable within the World Heath Organization classification, raise the possibility that CBL-MZ should be considered as a new provisional entity within the spectrum of clonal MZ disorders.

[Lymphocytic esophagitis: an entity to be excluded in chronic inflammatory diseases of the esophagus]. / Lymphozytäre Ösophagitis: Eine Ausschlussdiagnose in der Diagnostik chronischer Ösophagitiden.

Lymphocytic esophagitis is a rare, ill-defined inflammatory disease of the esophagus and is characterized by an increased number of intraepithelial lymphocytes. Up to now no distinct clinical symptom or endoscopic finding could be linked to histopathological changes. Hence lymphocytic esophagitis remains a diagnosis by exclusion after ruling out other possible causes of esophageal intraepithelial lymphocytosis.

Variant of lymphocytic infundibulo-neurohypophysitis presenting with unique clinical and radiological features.

Lymphocytic hypophysitis (LYH) is a chronic inflammation that primarily affects the pituitary gland. This disorder has recently been classified into lymphocytic adenohypophysitis (LAH), lymphocytic infundibulo-neurohypophysitis (LINH), and lymphocytic infundibulo-panhypophysitis (LIPH) according to the affected area. We report a case of LINH in a 68-year-old woman who presented with diabetes insipidus (DI). In this case, the posterior lobe was affected in both endocrinological assessment and magnetic resonance imaging (MRI) findings. In contrast, the anterior pituitary was not affected in endocrinological assessment but was affected in MRI findings. Indeed, the patient did not develop hypopituitarism. We believed that these clinical and radiological features were unique in regard to the classification of LYH. To confirm the classification of LYH and the distinction from pituitary adenoma, a pituitary biopsy was performed. Based on the pathological and endocrinological assessment, the patient's disorder was finally diagnosed as a variant of LINH. Current evidence recommends that surgical intervention for LYH should be avoided because the natural course of LYH is essentially self-limiting. Therefore, the accumulation of the knowledge of many variants of LYH is important for the preoperative differential diagnosis of pituitary masses. Our clinical observation could be useful for avoiding unnecessary surgical intervention.

Monoclonal B-cell lymphocytosis is closely related to chronic lymphocytic leukaemia and may be better classified as early-stage CLL.

The World Health Organization classification uses a cut-off point of 5·0 × 10(9)/l cells with a chronic lymphocytic leukaemia (CLL)-phenotype in peripheral blood to discriminate between monoclonal B-lymphocytosis (MBL) and B-CLL. This study analysed 298 MBL patients by multi-parameter flow cytometry, chromosome banding analysis (CBA)/fluorescence in situ hybridization (FISH), and IGHV mutation status and compared them with 356 CLL patients. In MBL, CBA more frequently revealed a normal karyotype and FISH identified less frequently del(6q), del(13q) (as sole alterations), and del(17)(p13). Within the MBL cohort, a shorter time to treatment (TTT) was found for ZAP-70-positivity, 14q32/IGH-translocations (CBA), del(11)(q22·3) (FISH) and unmutated IGHV status. Higher CD38 and ZAP-70 expression, del(11)(q22·3) (FISH), trisomy 12 (FISH), and 14q32/IGH-translocations (CBA) were correlated with a shorter TTT in the combined cohort (MBL + CLL); a sole del(13)(q14) (FISH) correlated with longer TTT. Regarding overall survival, unmutated IGHV status and 'other' alterations (CBA) had an adverse impact. There was no correlation between the concentration of CLL-cells and TTT or overall survival. Multivariate analysis confirmed a negative impact on TTT for del(11)(q22·3)/ATM, trisomy 12 (both by FISH), and 14q32/IGH-translocations by CBA. These data emphasize a close relationship between MBL and CLL regarding clinically relevant parameters and provide no evidence to strictly separate these entities by a distinct threshold of clonal B-cells.

Monoclonal B-lymphocytosis: demographics, nature and subclassification in 414 community patients.

We analyzed patients with small B-cell clonal populations in a non-hospital based pathology laboratory servicing metropolitan and regional areas of New South Wales, Australia. There were 414 patients with a finding of a B-cell clone with total B-lymphocytes < 5.0 × 10(9)/L, fulfilling the criteria for monoclonal B-lymphocytosis (MBL). There were 212 males (51%) and 202 females (49%) with a mean age of 69.7 years. Patients could be clearly divided into two dominant groups: 322 (77.7%) with a typical chronic lymphocytic leukemia (CLL) phenotype, MBL[cll], and 92 (22.3%) with a "non-CLL" or lymphoma-like phenotype, MBL[nhl]. Analysis of MBL[cll] showed 168 (52.2%) males and 154 (47.8%) females with a mean age of 70.6 years. The mean clonal level (CD19/CD5+) was 2.36 × 10(9)/L and the absolute lymphocyte count (ALC) was 0.4-10.5 × 10(9)/L. The ALC was within the reference range (1.0-4.0 × 10(9)/L) in 22%. The 92 patients with MBL[nhl] were 44 (47.8%) males and 48 (52.2%) females, with a mean age of 66.7 years. The mean clonal level was 1.27 × 10(9)/L. There were 65 patients with a "lymphoma unclassifiable" clone and the remainder had a probable disease-specific diagnosis. In a large community cohort of patients, MBL can be divided into two dominant groups, MBL[cll] and the more heterogeneous MBL[nhl].

Monoclonal B-cell lymphocytosis: definitions and natural history.

The diagnostic term monoclonal B-cell lymphocytosis (MBL) is used to characterise individuals with a circulating population of clonal B-cells, a total B-cell count of <5 x 10(9)/L, and no other features of a B-cell lymphoproliferative disorder. The clinical implications of MBL may differ depending on whether an individual with a normal lymphocyte count is identified via a screening assay (screening MBL) or identified through clinical evaluation of lymphocytosis (clinical MBL). The B-cell count used to distinguish between clinical MBL (<5 x 10(9)/L) and CLL (> or = 5 x 10(9)/L) was selected largely based on tradition and technological advances and it is unknown whether the natural history of 'clinical MBL' differs from that of patients with Rai stage 0 CLL. Since, a diagnosis of 'leukemia' may lead to profound psychologic distress for patients, we believe the diagnosis of CLL should be based on an individual's risk of developing symptoms, requiring chemotherapeutic treatment and/or dying of disease. Additional studies are needed to determine whether the clinical outcome of patients with MBL differs from that of patients with Rai stage 0 CLL and to identify what B-cell threshold optimally distinguishes between these conditions.

MBL or CLL: which classification best categorizes the clinical course of patients with an absolute lymphocyte count >or= 5 x 10(9) L(-1) but a B-cell lymphocyte count <5 x 10(9) L(-1)?

To eliminate overlap with monoclonal B-cell lymphocytosis (MBL), some have proposed basing the diagnosis of chronic lymphocytic leukemia (CLL) on B lymphocyte count rather than absolute lymphocyte count (ALC). Such criteria should be based, in part, on patient outcomes. We evaluated the clinical implications of the proposed re-classification in 112 consecutive, newly diagnosed, Rai stage 0 patients. The new criteria would have changed the diagnosis from CLL to MBL in 47/112 (42%) patients. There was no difference in time to treatment (TTT) between those classified as MBL and CLL under the new criteria. In contrast, CD38 predicted TTT (p=0.02) regardless of the proposed new classification. Molecular characteristics of the leukemic clone are a better predictor of progression than an arbitrary ALC or B lymphocyte count threshold.

Compromiso de las glándulas salivales en la enfermedad HIV/Sida / Salivary glands involvement in HIV/AIDS disease

La enfermedad de las glándulas salivales asociada al HIV es definida como la presencia de aumento del tamaño de la glándula asociado o no a xerostomía. Esta patología es más habitual en niños que en adultos. La enfermedad de las glándulas salivales asociada al HIV incluye lesiones linfoepiteliales y quistes que comprometen el tejido glandulas y/o los ganglios linfáticos, síndrome símil-Sjogren, síndrome de linfocitosis T CD8 difuso y neoplasias. Este artículo incluye una revisión de la epidemiología, la fisiopatología, las manifestaciones clínicas, la metodololgía diagnóstica y los diagnósticos diferenciales de la enfermedad de las glándulas salivales asociada al HIV (AU)