- About HSPC
Jose Osvaldo Barbosa Neto*, Joao Batista Santos Garcia, Thiago Alves Rodrigues and Camila Freitas de Andrade Rodrigues
Introduction: Erdheim-Chester disease (ECD) is a rare and diffi cult-to-treat non-Langerhans cell histiocytosis characterized by the excessive production and accumulation of histiocytes. This study reports a case of ECD, emphasizing both its diagnosis, assessment and treatment of the
pain associated with the disease.
Case Report: Six years ago, a 39-year-old male patient presented with generalized pain of moderate intensity in the lower limbs that involved periods of greater intensity associated with ambulation. The diagnosis of histiocytosis associated with panhypopituitarism and adrenal insufficiency was proposed. For a specific diagnosis, a bone lesion biopsy was performed, revealing the presence of histiocytic proliferation that was CD1 negative, S100 protein positive, and CD68 negative. Therefore, the diagnosis of non-Langerhans histiocytosis known as ECD was confirmed. During the two years that followed, the patient presented with severe bone pain, particularly in the lower limbs and cranial vault, and the pain subsided to a certain extent with the use of tramadol and paracetamol. Because of the pain, the patient was unable to walk and became bedridden As the patient remained in severe pain, even after the administration of morphine, the opioid was changed from morphine (60mg/day) to oxycodone (30mg/day) for a convenient dosing schedule; furthermore, the oxycodone dosage was scheduled to increase to 40mg/day that same week. The patient experienced significant pain reduction, requiring rescue analgesia only once or twice a week.
Conclusion: To the best of our knowledge, this is the first case report on the characterization and treatment of pain specific to ECD, and we highlight that the patient had a good response to treatment.
Vincenzo Accurso*, Santoro M, Contrino AD, Casimiro P, Raso S, Carlisi M, Sardo M, Perez A, Di Piazza F, Russo A and Siragusa S
Essential Thrombocythemia (ET) is currently classified as a Philadelphia negative myeloproliferative neoplasm (MPN) together with polycythemia vera (PV) and primary myelofibrosis (PMF); the latter can be further divided in pre-fibrotic primary myelofibrosis (pre-PMF) and overt myelofibrosis, as listed in the revised 2016 World Health Organization classification of myeloid malignancies (WHO 2016). Overall, respect to the others MPNs, ET is characterized by favorable prognosis, lower life expectancy if compared to the control population, increased risk of thrombohemorrhagic complications along with possible evolution in myelofibrosis and leukemic transformation. In this review the authors will review current knowledge on biology, clinical aspects, prognosis and stratification of thrombotic risk, therapeutic options and outcome in ET patients.
Muhammad Imran Qadir and Sani E Zahra*
In 1901, ABO system of blood groups was determined by Karl Landsteiner. It is present in different organisms like rodents, primates that includes chimpanzees, bonobos etc. The blood groups type in this system depends on some genes that are specific ABO gene. Arachnophobia is the dread of creepy crawlies and different creature like spiders. Individuals suffering from arachnophobia generally feels uneasy in any place where they accept that they could harbour arachnids or their existence for example, webs. 100 samples of blood from volunteers were used for Blood group test. The Blood groups were tested and results were recorded after the test all the used kits were discarded. The results shows that there is no clear cut difference between the arachnophobic males and non-arachnophobic male’s percentages, so no relation found in males. Similarly, in females both phobic and non-phobic ladies have no differences in their result values so, no relation was found. Whereas, in case of arachnophobic males and females comparison no relation was found. So, there is no relationship between ABO blood group system and arachnophobia and there may be a relation between non-phobia and AB+, B+ blood group in males whereas, in females only B+ blood group have relation with non-phobia.
Khalid Ahmed Al-Anazi*, Al-Anazi WK and Al-Jasser AM
Varicella zoster virus behaves differently from other herpes viruses as it differs from them in many aspects. Recently, there has been growing evidence on the beneficial effects of the virus in immune compromised hosts and these effects are translated into prolongation of survival. The reported beneficial effects of the virus include: (1) stimulation of bone marrow activity in patients with hematologic malignancies and bone marrow failure syndromes, (2) antitumor effects in various hematologic malignancies and solid tumors, and (3) association with graft versus host disease which has anticancer effects. Additionally, there are several reports on the safety of the live-attenuated even in severely immune suppressed individuals and on the emerging role of the virus in cancer immunotherapy. In this review, the following aspects of the virus will be thoroughly discussed: (1) new data on the genetic background, pathogenesis, vaccination, and new therapeutic modalities; (2) bone marrow microenvironment and hematopoiesis; (3) cells involved in the pathogenesis of the virus such as: mesenchymal stem cells, dendritic cells, natural killer cells, T-cells and mononuclear cells; (4) cellular proteins such as open reading frames, glycoproteins, promyelocytic leukemia protein, chaperons, and SUMOs; (5) extracellular vesicles, exosomes, and micro-RNAs; and (6) signaling pathways, cytokines, and interferons.
Al-Anazi KA* and Al-Jasser AM
Varicella zoster virus (VZV), a double-stranded DNA virus, is a highly contagious human neurotropic virus that belongs to the alpha group of herpes viruses [1-4]. Primary VZV infection (chickenpox) occurs in childhood then the virus becomes latent in the nerve ganglia [1,5-7]. Reactivation of the virus may occur decades later and cause herpes zoster (HZ) which is manifested by a typical painful skin eruption that has characteristic dermatomal distribution [1,5]. Reactivation of VZV is usually predisposed to: old age; comorbid medical conditions such as diabetes mellitus, chronic obstructive airway disease, and end-stage renal disease; and immunosuppression due to malignancy, autoimmune disorders, immunosuppressive therapies, trauma, cytotoxic chemotherapy, hematopoietic stem cell transplantation (HSCT), and solid organ transplantation (SOT) [1,5-7].
VZV infections can cause not only transient pancytopenia but also aplastic anemia that may require allogeneic HSCT [8-12]. Additionally, VZV infections have been reported to be associated with increased risk of developing lymphoid malignancies and solid tumors [13-17]. On the contrary, there is growing evidence showing certain beneficial effects of the virus in immunocompromised individuals and these effects may be translated into prolongation of overall survival (OS) [18,19]. In a single center, case-controlled, retrospective study that included 16 episodes of VZV infections occurring in 14 patients with various types of hematologic malignancies (HMs) and bone marrow (BM) failure syndromes, Al-Anazi KA et al., reported an increase in white blood cell count, hemoglobin (Hb) level, and platelet count starting approximately 6 weeks following VZV infections and this stimulation of the 3 hematopoietic cell lines in the BM that followed VZV infections was maintained for periods longer than 3 years following the infection . In another single center retrospective study that included a large number of patients with multiple myeloma subjected to high-dose melphalan followed by autologous HSCT after control of their primary disease, Kamber C et al., reported that approximately one third of these patients developed VZV infections either before or after HSCT . Despite encountering VZV infections in patients with worse expected prognosis, the OS in patients who developed VZV infection was superior to that in patients who never developed the infection . Recently, Al-Anazi KA et al., reported BM biopsy-proven reversal of pure red cell aplasia manifested by a gradual increase in Hb level starting 6 weeks following a localized HZ infection till the Hb level plateaued above 14g/dL fourteen months following the viral infection . Additionally, several studies have demonstrated that VZV infection may trigger chronic graft versus host disease (GVHD) following allogeneic HSCT [20-22]. GVHD is associated with graft versus cancer effects and, provided GVHD is of low-grade, it can be associated with improvement in OS in patients with acute leukemia or lymphoma [23-25].
Al-Anazi KA*, Kanfar S, Aldayel A, Abduljalil O and Sayyed AH
Background: Pure red cell aplasia is characterized by anemia, reticulocytopenia and diminished bone marrow erythroid precursors. It has multifactorial etiology and consequently several therapeutic interventions.
Case: In August 2017, a young patient was diagnosed to have pure red cell aplasia. She was given immunosuppressive therapy for approximately two months but this treatment was stopped due to intolerance. Later on she developed herpes zoster infection that was treated with valacyclovir. Subsequently, it was noted that the patient became blood transfusion independent due to normalization of her hemoglobin and regeneration of the erythroid precursors in the bone marrow.
Discussion: Varicella zoster virus behaves differently from other members of the herpes group of viruses such as cytomegalovirus and Epstein-Barr virus. Two retrospective studies, performed in patients with malignant hematological disorders and bone marrow failure, have shown that infection with the virus may cause stimulation of the three cell lines in the bone marrow and superior overall survival.
Conclusion: The outcome of the patient presented confirms the findings of the two studies showing long-term beneficial effects of varicella zoster virus infections in immunocompromised individuals.
AlShammasi S, AlNujaidi D, Bakhit K, Algarni A and AlAnazi KA*
Background: Invasive fungal infections cause significant morbidity and mortality in patients with hematologic malignancies and in recipients of hematopoietic stem cell transplantation.
Case: We report a patient with relapsed acute myeloid leukemia who developed disseminated Fusarium infection during the neutropenic period following the salvage cycle of chemotherapy given at King Fahad specialist Hospital in Dammam, Saudi Arabia. The invasive fungal infection was successfully managed with a combination of voriconazole and liposomal amphotericin-B.
Discussion: Fusarium species can cause invasive infections that may become disseminated and life-threatening in patients with acute myeloid leukemia.
Conclusion: Combined antifungal therapy and recovery of neutrophil count are essential to control invasive Fusarium infections
Hausatu Babayi*, Ojo Olufunmilola Praise, Labake Fadipe Ajoke, Israel Kayode Olayemi, Elisha Baba and Oladosun Oluwale Peter
The effects of Leech Salivary Extract (LSE) on some haematological, immunological and organ weight parameters in rats, during a twenty eight days oral administration of 25, 50 and 100 mg/kg body weight doses, was investigated. LD50 and sub chronic toxicity was determined using standard methods. The oral LD50 was above 5000mg/kgbw. Oral administration of LSE (25mg/kgbw, 50mg/kgbw, 100mg/kgbw) for 28days had no significant (p>0.05) effect on the differential white blood cells (lymphocytes, monocytes, basophils, neutrophils, eosinophils), red blood cell indices (RBC count, PCV, HB, platelets, MCHC and MCH), feed intake, body weight gain and relative organ weight of lung, heart, liver, kidney, spleen and stomach of rats. However, the LSE evoked a significant (p>0.05) increase in the level of MCV in treated rats compared to the control. These results, indicating low toxicity and no negative significant effects of LSE on haemato-immunological indices in rats, suggest that the extract is safe for development and use as therapeutic for managing clinical conditions.
Pandey Prashant, Agarwal Nitin*, Das Suryasanta and Dharmendra Kumar
Background and Aims: the Aim of the study was to find the level of protection among the healthcare workers (nurses, doctors, housekeeping staff and general duty assistants) by doing Anti-HBsAb titer and vaccinate those who were not properly immunized against HBV infection.
Materials and Methods: The study was approved by the Institutional review board of the Hospital. The study group included doctors, nurses, technical staff and lab attendants. Anti-HBs antibody titer was done on Vitros 3600 (OCD, USA). Tests were performed according to manufacturer’s instruction. Vaccine provided was Engerix B (GSK Glaxo, Belgium). Vaccination was provided to all employees had titer below 10 miu/ml.
Results: 489 of 794(61.5%) HCW had no history of previous vaccination and only 293 (36.9%) subjects had complete vaccination. Only 60.8 % (482/794) of the total subjects had titer above 10 miu/ml and were protected against Hepatitis B. Around 80.6% (246/305) of those who were fully vaccinated and 40.8% (237/489) of those who were not vaccinated previously had protective anti-HBs titers(>10 miu/ml). Majority (86.8%, 271/312) who had titer below 10 miu/ml were unvaccinated. Two of eight employees who had history of needle stick injury in past were found non-immune to Hepatitis-B infection.
Conclusion: Despite being involved in the procedures with high chances of infections through needle stick or other exposures, only one third of health care workers were vaccinated against hepatitis B. We recommend that all the HCWs should be vaccinated for Hepatitis B and their anti-HBs levels determined at regular intervals.
Mark IM Noble* and Angela J Drake-Holland
An enquiry into the lack of attention awarded to serotonin antagonism in the treatment of arterial thrombosis revealed that the mode of action of serotonin and its platelet receptor antagonists was an action upon thrombus growth, and not, as with other anti-platelet drugs upon the initiation of thrombosis. This lack of effect could explain why this approach has been considered not to be effective. However under conditions of arterial stenosis in which there is platelet activation by increased shear stress, and during the growth phase of arterial thrombi, serotonin 5HT2A antagonism has been demonstrated to have great potentcy in dispersing thrombotic obstruction to blood flow. This mode of action, the lack of participation of serotonin in haemostasis, and the absence of serotonin in wounds accounts for the proven lack of effect of effect of pure specific 5HT2A antagonists (i.e., not those with other actions) on operative bleeding and skin bleeding times. This lack of effect on haemostasis solves the dosing problem encountered with other anti-thrombotic drugs, with which drug concentration cannot be controlled with single fixed doses, leading to the association between increased anti-thrombotic efficacy and increased bleeding complications. Thus 5HT2A antagonism appears to be the preferred approach, from the point of view of safety and lack of bleeding risk; this consideration applies particularly to thrombosis therapy in the context of traumatic accidents, surgical operations and invasive procedures such as angioplasty.
Zuhre Kaya*, Tuba Sismanlar, Ayse Tana Aslan, Oznur Konus Boyunaga, Isıl Fidan Balcı, İdil Yenicesu and Ulker Kocak
Background: Knowledge of pulmonary complications (PCs) in children after hematopoetic stem cell transplantation (allo-HSCT) is limited; most data are from adult studies.
Case: We describe a 8 year old girl with high risk acute myeloid leukemia who developed graft versus host disease (GVHD) on Day 20, Cytomegalovirus (CMV) pneumonia on Day 50 and Cryptogenic organizing pneumonia (COP) on Day 170 after allo-HSCT.
Discussion: Cryptogenic organizing pneumonia is a rare noninfectious PCs that can be idiopathic or have several risk factors as a secondary causes, such as viral respiratory infections, drugs, GVHD and allo-HSCT. Viral respiratory infections and alloimmune lung syndromes have been reported in a few patients who have undergone transplantation.
Conclusion: Transplant physicians should be kept in mind for the development of alloimmune lung syndrome in the form of COP following CMV pneumonia in patients after allo- HSCT
Xiaoli Zhang, Ziyuan Zou, Liyu Fan, Xinjie Xu, Yu Siyuan and Peng Luo*
Lung cancer is the leading cause of cancer-related deaths worldwide, and almost accounts for 20% of these deaths, however, the cure rate is less than 10% . Non-small cell lung cancer (NSCLC) accounts for approximately 85% of all cases of lung cancer , but fewer than 15% of individuals diagnosed with NSCLC can survive for more than 5 years, which poses a great threat to the patient’s life and health . Recently, the incidence of lung cancer keeps dynamically growing, but more than 75% of patients at diagnosis has appeared local development or metastasis, missing the best period of surgery. Moreover, despite surgical treatment is the optimal choice for early-stage NSCLC patients, 30%-40% of patients with NSCLC develop tumor recurrence in a short time. Therefore, improving the prognosis of patients with lung cancer and predicting the long-term survival of patients is of particular importance . At present, tumor and node metastasis (TNM) staging system, clinicopathological characteristics, visceral pleural invasion and marginal status are used to predict the disease progression and overall survival of NSCLC patients. There is no index which is stable, effective, reliable and less harmful to assess prognosis, predict recurrence risk and overall survival.
The genesis, progress and metastasis of NSCLC are not only related to the intrinsic characteristics of cancer cells but also to the cancer microenvironment. Systemic inflammation is a promoter of tumor genesis. Cumulative evidence has revealed that tumor-promoting inflammation contributes to cancer growth and progression by creating a tumor-promoting microenvironment, stimulates the proliferation of bronchial stem cells, and triggers the carcinogenesis of lung epithelial cell. Additionally, systematic inflammatory can not only cause tumor growth and promote tumor metastasis by activating type 2 T-assisted response, chemokines, vascular regeneration and others , but also responsible for reducing the effect of anti-cancer drugs and cancer-related symptoms including anorexia, cancerous pain, debilitation, cachexia . In total, systematic inflammation has been shown to play an important impact on the pathogenesis and progression of NSCLC and is of great value to the aggravation and prognosis of NSCLC.
Ajda ERSOY GUNES, Melda COMERT OZKAN*, Fahri SAHIN and Guray SAYDAM
Primary cutaneous lymphomas (PCLs) are the second most common group of extranodal non-Hodgkin lymphomas (NHL) with an estimated annual incidence of 1/100.000. Interferons (IFNs) are used in mono or combination therapy for cutaneous lymphomas especially for cutaneous T-cell lymphomas (CTCL) for years. IFN-α is the most widely-used type for cutaneous lymphomas. IFN-α has been shown to be a highly active agent in CTCL with response rates ranging from 40% to 80%. In this review, the current information about PCLs and IFNs treatment is summarized.