Case Report A Girl with Autoimmune Cytopenias, Nonmalignant Lymphadenopathy, and Recurrent Infections

We describe a girl, now 9 years of age, with chronic idiopathic thrombocytopenic purpura, persistent nonmalignant lymphadenopathy, splenomegaly, recurrent infections, and autoimmune hemolytic anemia. Her symptoms partly fit the definitions of both autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency disorders (CVIDs). Genetic analysis showed no abnormalities in the ALPS-genes FAS, FASLG, and CASP10. The CVID-associated TACI gene showed a homozygous polymorphism (Pro251Leu), which is found also in healthy controls

Dynamics of peripheral blood lymphocyte subpopulations in the acute and subacute phase of Legionnaires' disease

Absolute lymphocytopenia is recognised as an important hallmark of the immune response to severe infection and observed in patients with Legionnaires' disease. To explore the immune response, we studied the dynamics of peripheral blood lymphocyte subpopulations in the acute and subacute phase of LD. EDTA-anticoagulated blood was obtained from eight patients on the day the diagnosis was made through detection of L. pneumophila serogroup 1 antigen in urine. A second blood sample was obtained in the subacute phase. Multiparametric flow cytometry was used to calculate lymphocyte counts and values for B-cells, T-cells, NK cells, CD4+ and CD8+ T-cells. Expression of activation markers was analysed. The values obtained in the subacute phase were compared with an age and gender matched control group. Absolute lymphocyte count (×10⁹/l, median and range) significantly increased from 0.8 (0.4-1.6) in the acute phase to 1.4 (0.8-3.4) in the subacute phase. B-cell count showed no significant change, while T-cell count (×10⁶/l, median and range) significantly increased in the subacute phase (495 (182-1024) versus 979 (507-2708), p = 0.012) as a result of significant increases in both CD4+ and CD8+ T-cell counts (374 (146-629) versus 763 (400-1507), p = 0.012 and 119 (29-328) versus 224 (107-862), p = 0.012). In the subacute phase of LD, significant increases were observed in absolute counts of activated CD4+ T-cells, naïve CD4+ T-cells and memory CD4+ T-cells. In the CD8+ T-cell compartment, activated CD8+ T-cells, naïve CD8+ T-cell and memory CD8+ T-cells were significantly increased (p <0.05). The acute phase of LD is characterized by absolute lymphocytopenia, which recovers in the subacute phase with an increase in absolute T-cells and re-emergence of activated CD4+ and CD8+ T cells. These observations are in line with the suggested role for T-cell activation in the immune response to L

The Neutrophil-Lymphocyte Count Ratio in Patients with Community-Acquired Pneumonia

Study Objective: The neutrophil-lymphocyte count ratio (NLCR) has been identified as a predictor of bacteremia in medical emergencies. The aim of this study was to investigate the value of the NLCR in patients with community-acquired pneumonia (CAP). Methods and Results: Consecutive adult patients were prospectively studied. Pneumonia severity (CURB-65 score), clinical characteristics, complications and outcomes were related to the NLCR and compared with C-reactive protein (CRP), neutrophil count, white blood cell (WBC) count. The study cohort consisted of 395 patients diagnosed with CAP. The mean age of the patients was 63.4 +/- 16.0 years. 87.6% (346/395) of the patients required hospital admission, 7.8% (31/395) patients were admitted to the Intensive Care Unit (ICU) and 5.8% (23/395) patients of the study cohort died. The NLCR was increased in all patients, predicted adverse medical outcome and consistently increased as the CURB-65 score advanced. NLCR levels (mean +/- SD) were significantly higher in non-survivors (23.3 +/- 16.8) than in survivors (13.0 +/- 11.4). The receiver-operating characteristic (ROC) curve for NLCR predicting mortality showed an area under the curve (AUC) of 0.701. This was better than the AUC for the neutrophil count, WBC count, lymphocyte count and CRP level (0.681, 0.672, 0.630 and 0.565, respectively). Conclusion: Admission NLCR at the emergency department predicts severity and outcome of CAP with a higher prognostic accuracy as compared with traditional infection marker

Relative expansion of lymphocyte subpopulations in the subacute phase compared to the acute phase of Legionnaires’ disease.

<p>RE, relative expansion: relative decrease or increase of the different absolute lymphocyte subpopulation counts in the acute versus the subacute phase compared to the relative increase of the absolute lymphocyte count in the same period; all data presented as mean and standard deviation; NK, natural killer cells; *Wilcoxon Signed Rank tests, significant difference p-value <0.05.</p

Flow cytometric analysis of activated CD4⁺ T-cells (top) and activated CD8⁺ T-cells (bottom) in the acute (left) and subacute phase (right) in one Legionnaires’ disease patient.

<p>Activated T-cells are defined as CD38+ and HLA-DR+ double positive cells and located in the upper right quadrants of each dot plot. The numbers represent the percentage of cells in the upper right quadrant.</p

Baseline characteristics upon hospitalization of Legionnaires’ disease patients (n = 8).

<p>NA, not applicable; M, male, F, female; Temp, temperature (C) upon presentation to the ED; CRP, C-reactive protein (mg/l); WBC, white blood cell (10⁹/l); lymphocyte count expressed as 10⁹/l; X-ray, chest radiography results upon presentation to the ED; AB, adequate antibiotics within 12 hours after presentation; Duration of symptoms before ED presentation, ICU, Intensive Care Unit admission, LOS, length of stay.</p

CRP levels, WBC counts, absolute neutrophil -, absolute lymphocyte - and lymphocyte subpopulation counts in the acute and subacute phase of Legionnares’ disease (n = 8) and in age and gender matched healthy controls (n = 8).

<p>NA, not applicable; LD, Legionnaires’ disease; CRP, C-reactive protein (mg/l); WBC, white blood cell (10⁹/l); absolute neutrophil count expressed as 10⁹/l; NK, natural killer; absolute lymphocyte count expressed as 10⁹/l; lymphocyte subpopulation counts expressed as 10⁶/l; all data presented as median and range; *p-value Wilcoxon Signed Rank tests for differences in CRP levels, WBC counts, absolute neutrophil, absolute lymphocyte and lymphocyte subpopulation counts between the acute and subacute phase of LD; <b>^#</b>p-value Mann Whitney U tests for differences between the subacute phase of LD and healthy controls.</p

Baseline characteristics upon presentation at the Emergency Department (n = 395).

<p>COPD, chronic pulmonary obstructive disease; Chronic Obstructive Pulmonary Disease (COPD) was classified using the global initiative for chronic obstructive lung disease classification (GOLD), Hepatic means liver disease related to malignancy, hepatitis, auto-immune liver disease and/or alcoholic liver disease, cardiac means heart disease related to acute coronary syndrome (cardiovascular disease), valvular disease and/or heart failure, renal means renal disease including current renal replacement therapy, cerebrovascular means cerebrovascular disease; n, number; ICU, intensive care unit; data are presented as number (percentage) of patients.</p