Primary immune regulatory disorders (PIRDs) are a group of diseases belonging to inborn errors of immunity. They usually exhibit lymphoproliferation, autoimmunities, and malignancies, with less susceptibility to recurrent infections. Unlike classical primary immune deficiencies, in autoimmune manifestations, such as cytopenias, enteropathy can be the first symptom of diseases, and they are typically resistant to treatment. Increasing awareness of PIRDs among specialists and a multidisciplinary team approach would provide early diagnosis and treatment that could prevent end-organ damage related to the diseases. In recent years, many PIRDs have been described, and understanding the immunological pathways linked to these disorders provides us an opportunity to use directed therapies for specific molecules, which usually offer better disease control than known classical immunosuppressants. In this review, in light of the most recent literature, we will discuss the common PIRDs and explain their clinical symptoms and recent treatment modalities.

Objective: The defective interplay between coagulation and inflammation may be the leading cause of intravascular coagulation and organ dysfunction in coronavirus disease-19 (COVID-19) patients. Abnormal coagulation profiles were reported to be associated with poor outcomes. In this study, we assessed the prognostic values of antithrombin (AT) activity levels and the impact of fresh frozen plasma (FFP) treatment on outcome.
Materials and Methods: Conventional coagulation parameters as well as AT activity levels and outcomes of 104 consecutive critically ill acute respiratory distress syndrome (ARDS) patients with laboratoryconfirmed COVID-19 disease were retrospectively analyzed. Patients with AT activity below 75% were treated with FFP. Maximum AT activity levels achieved in those patients were recorded.
Results: AT activity levels at admission were significantly lower in nonsurvivors than survivors (73% vs. 81%). The cutoff level for admission AT activity was 79% and 58% was the lowest AT for survival. The outcome in those patients who had AT activity levels above 75% after FFP treatment was better than that of the nonresponding group. As well as AT, admission values of D-dimer, C-reactive protein, and procalcitonin were coagulation and inflammatory parameters among the mortality risk factors.
Conclusion: AT activity could be used as a prognostic marker for survival and organ failure in COVID-19-associated ARDS patients. AT supplementation therapy with FFP in patients with COVID-19-induced hypercoagulopathy may improve thrombosis prophylaxis and thus have an impact on survival.

Objective: Fresh frozen plasma (FFP) transfusion is widely used in modern clinical settings. Practices regarding its use vary due to lack of guidelines from randomized trials. The aim of this study was to assess both the current practices regarding FFP production, use, and wastage and the implementation of quality control (QC), female donor plasma production policies, and use of pharmaceutical hemostatic agents in Greece.
Materials and Methods: The study was conducted during February-April 2018. For the first part of the study, data including FFP transfusion indication, hospital department, diagnosis, FFP units/transfusion episode, ABO compatibility, blood donor’s sex, and reasons for discarding were collected. For the second part, questionnaire data were analyzed.
Results: According to data from 20 Greek hospitals, 12655 FFP units were transfused to 2700 patients during 5069 transfusion episodes in the studied period of time. Most patients were hospitalized in internal medicine, general surgery, and intensive care unit departments. Each patient received on average 4.69 units (2.5 units/episode). Transfusion requests were in accordance with international guidelines in 63.44% of cases and 99.04% of the units were given to ABO-identical patients. Main reasons for discarding included failure to meet quality requirements (30.06%), female donors (22.17%), and other causes (27.26%). Among 96.9% of all transfusion services across the country, 28.26% perform QC according to the directions of the European Directorate for the Quality of Medicines & Health Care and 68.83% discard plasma from female donors. Pharmaceutic hemostatic agents are used in 37.23% of the hospitals.
Conclusion: This is the first national survey regarding FFP production and transfusion in Greece. Staff of internal medicine, general surgery, and ICU departments, where most FFP-transfused patients are hospitalized, should be regularly involved in training on contemporary transfusion guidelines. Upcoming centralization of FFP production and inventory management could help in homogenizing practices regarding FFP use and improve product quality. Strengthening the use of pharmaceutic hemostatic agents could improve patients’ management.

Objective: The prognosis of multiple myeloma (MM) patients is highly heterogeneous. The aim of this study is to determine the impact of patients’ renal functions on the prognostic performance of the International Staging System (ISS). In addition, we aimed to evaluate the results of survival of patients with ISS stages and normal renal functions and those with ISS stages and abnormal renal functions with this study.
Materials and Methods: Two hundred and four patients with newly diagnosed MM who received an autologous stem cell transplantation after induction chemotherapy in our tertiary care center between the years of 2001 and 2018 were evaluated.
Results: There were 153 (75%) MM patients who had a glomerular filtration rate (GFR) of ≥60 mL/min and 51 (25%) MM patients who had GFR of <60 mL/min at the time of diagnosis in this study. There was a strong correlation between ISS stage and GFR. The ISS stages were higher in patients who had GFR of <60 mL/min than patients who had GFR of ≥60 mL/min (p<0.001). Patients with GFR of <60 mL/min were significantly more prevalent in the ISS III group than ISS I and II (p<0.001).
Conclusion: This study showed that the ISS provides significant prognostic information in MM patients with GFR of ≥60 mL/min at diagnosis. However, in patients with impaired renal function at the time of diagnosis, B2-microglobulin may not be a good prognostic indicator since it may be affected by renal dysfunction as well as tumor burden.

Objective: Lenalidomide is an effective immunomodulatory derivative drug used in the treatment of multiple myeloma (MM). It is available in original and generic forms in Turkey, but there is no clinical study that has compared the effectiveness and adverse events (AEs) of the generic and original forms of lenalidomide. We compared the effectivity and AEs of generic and original lenalidomide in patients with relapsed/refractory MM (RRMM).
Materials and Methods: Patients with RRMM using original or generic lenalidomide were evaluated retrospectively. Overall response (OR), complete response (CR), very good partial response (VGPR), partial response (PR), stable disease, and progressive disease rates and hematologic and nonhematologic AEs were evaluated in these RRMM patients. The results were described as numbers, frequencies, and percentages and were analyzed using PASW 19.0 for Windows with chi-square and Fisher exact tests.
Results: The number of patients using original lenalidomide was 55 and the number of patients using generic lenalidomide was 43. The OR rate was 67.2% for patients using original lenalidomide and 60.4% for those on generic lenalidomide. CR and VGPR rates were 14.5% and 45.4% in the original group while the CR and VGPR rates were 20.9% and 18.6%, respectively, in patients using generic lenalidomide. Hematologic AEs were similar in the two groups while some nonhematologic AEs were less common in the original lenalidomide group than the generic group. Only pyrexia as a grade 3-4 AE was more common in the original lenalidomide than the generic lenalidomide group.
Conclusion: This study showed that the generic form of lenalidomide has similar efficacy with the original form of lenalidomide in the treatment of RRMM. The AEs of original lenalidomide were generally fewer than those of generic lenalidomide. Further studies involving a larger number of patients with RRMM would be useful for comparing the efficacy and AEs of original and generic lenalidomide.

Objective: Lymphomatoid papulosis (LyP) is an indolent skin disease with variable clinical features classified among the primary cutaneous CD30+ T-cell lymphoproliferative disorders. It may show association with cutaneous and systemic lymphomas. We aimed to identify the frequency and characteristics of associated lymphomas among Turkish patients with LyP and to determine the risk factors for secondary lymphomas.
Materials and Methods: The files of patients diagnosed with LyP between 1998 and 2018 in a tertiary dermatology clinic were retrospectively analyzed. Univariate and multivariate models were used to assess the possible risk factors for secondary lymphomas, such as demographic and clinical characteristics of the patients.
Results: Among 61 patients (47 adults, 14 children) with LyP, a total of 22 secondary lymphomas were observed in 20 patients. Nineteen of them were adults. Mycosis fungoides (MF) was the major associated lymphoma (n=19) followed by systemic anaplastic large cell lymphoma (ALCL) (n=2) and primary cutaneous ALCL (n=1). The most common stage in patients with accompanying MF was stage IB (n=11). While 18 patients showed the classical type of MF, one patient had folliculotropic MF. When the risk factors for association between LyP and other lymphomas were evaluated, only older age was found to be a significant risk factor and existence of ulcerated lesions was found to be a negative indicator.
Conclusion: LyP is not rare in the pediatric population. MF is the most common associated lymphoma in patients with LyP. Adult LyP patients are more commonly associated with secondary lymphomas than pediatric patients. Older age at the time of diagnosis of LyP is a significant risk factor for associated lymphomas.

Objective: Febrile neutropenia (FN) is a hematological emergency requiring urgent investigations to exclude infection and treatment with broad-spectrum antibiotics. Despite frequent blood cultures (BCs) being taking during episodes of FN, in the current literature BC positivity rates remain low in FN. This study aims to determine the BC positivity rate in FN hematology patients and determine the utility of collecting BCs beyond 24 h of commencing broad-spectrum antibiotics.
Materials and Methods: BC results between 2014 and 2016 from all FN hematology patients were analyzed. Patient episodes of FN (PEFNs) were defined as a continuous period of FN where the interval between BC samples was a maximum of two days. In total from 2014 to 2016, 379 patients experienced 914 PEFNs and had 4267 BCs collected.
Results: Overall BC positivity rates and BC-positive PEFN rates were 8.16% and 13.35%, respectively. Within the first 24 h, the positivity rate of the first BCs was 3.49%, while subsequent BC positivity within the first 24 h was 11.96%. BC positivity rates declined after 24 h to 2.18%.
Conclusion: It is likely that BCs beyond 24 h of commencing broadspectrum antibiotics will rarely identify relevant microorganisms. Not collecting BCs after 24 h would likely reduce laboratory test costs, patient discomfort, and iatrogenic anemia.

Objective: Several clinical scoring systems have been developed for the differential diagnosis of thrombotic microangiopathies (TMAs), all to predict and identify patients with ADAMTS13 deficiency and to start treatment as soon as possible. The first scoring system in this regard was the Bentley score, and the French score and PLASMIC score were developed afterwards.
Materials and Methods: We aimed to evaluate the laboratory parameters and clinical features of patients who underwent plasma exchange with a prediagnosis of TTP at our clinic between 2007 and 2019 and whose ADAMTS13 enzyme levels were measured and to compare the findings with the scoring systems.
Results: Data of 35 patients were evaluated. Twelve patients were evaluated as high risk according to all three scoring systems. A statistically significant relation was observed between all three scoring systems and ADAMTS13 levels.
Conclusion: A moderate correlation was found between all three scoring systems and ADAMTS13 levels. We observed similar potential strength of all three scoring systems to predict TTP among other TMAs and we conclude that they are applicable in daily practice.