Journal of Blood & Lymph

Composite lymphoma is a rare entity of lymphoma where two types of lymphoma are present synchronously. We recently encountered two cases of composite lymphomas that are not rare in the morphology and pathology only, but also rare in the site of involvement and presentation. In this report we present a case of composite lymphoma that presented as primary splenic lymphoma, the second case presented with GI symptoms due to its presence in the colon.

Blood group diet research is gaining popularity among scientists and they are exploring new reasons to preferences of diet in four blood group type individuals. The concept of balanced diet and nutrition seems not to be working properly because diseases like obesity, diabetes, CVD, and cancer are causing millions of deaths each year in the world. Many scientists still did not pay any attention to the strong correlation between blood group diet and diseases except few in the world. There are strong evidences that these four blood group individuals have different taste buds which are the bases for selection of foods which ultimately become nutrition of that individual. A very nutritious food if not selected by a person having a particular blood group will provide no any nutrition to that particular individual. Blood group “A” has bland, “B” has sweet, “O” has saltish, and “AB” has bitter and astringent taste buds. Distribution of blood group types in different regions of the world indicates that there are strong variations in blood group diet because all four blood group types have four different types of tissues (A- nervous, B-epithelial, O-muscular and AB-connective). Macro and micronutrients are also specific to these blood group types (A-Zinc & Magnesium, B-Iron, O-Iodine and AB need additional calcium). Pakistan has blood groups population as “B” 36%, “O” 33%, “A” 21%, and “AB” 9%. USDA diet pyramids were designed to guide about the diet of Human beings living in different regions of the world. But these diet pyramids are no more valid because of the reason that they are nutritionally and biochemically unsound, but still in many countries these pyramids are being used for the assessment of diet without any positive effects. A diet pyramid based on blood groups is designed to guide about the diet of individuals based on blood groups. Diet charts formulated for four blood group types are based on scientific correlation to prevent diseases and remain healthy.

Wernicke-Korsackoff Syndrome is a potentially fatal neurological complication caused by a deficiency of thiamine. The clinical diagnosis is a challenge and the syndrome is frequently misdiagnosed. In this paper we describe the case of a young man affected by T-ALL and ileotyphlitis who developed Wernicke’s Encephalopathy after a month of total parenteral nutrition. Neurological symptomatology, consisting of confusion, nystagmus on lateral gaze, impairment of consciousness state, prosopagnosia and weakness in the four limbs, appeared progressively. An MRI of brain permitted to confirm the diagnosis and high-dose thiamine reintegrating therapy was started obtaining complete resolution of the syndrome.

Background: Autoimmune sensitization by antihypertensive drugs is an emerging cause of morbidity and mortality among hypertensive on antihypertensive therapy and little is known about it in our environment. The aim of this study is to evaluate autoimmune sensitization among hypertensive patients on antihypertensive therapy using DAT (Direct Antiglobulin test or Direct Coomb’s test) and Hemoglobin concentration.
Materials and method: This was a 4-month single center prospective study of 60 hypertensive patients on antihypertensive drugs and 40 age and sex matched control who were attending Cardiology Clinic in BMSH Port Harcourt. Hemoglobin concentration, Packed Cell Volume (PCV) and DAT were obtained using Cyanmethemoglobin, Microhematrocrit and Conventional tube methods respectively, while other clinical data were obtained using structured questionnaires. Data analysis was by SPSS version 20.
Results: There was no significant change in the mean hemoglobin concentration/PCV of the hypertensive patients on therapy and their control counterparts (13.4 g/dL/40.7% versus 13.4 g/dL/40.7%; p>0.05). The DAT in the hypertensive patients on therapy and their hypertensive control counterparts was negative.
Conclusion: Although antihypertensive drugs have the tendency of instigating immune sensitization of red blood cells, this was not demonstrated from this study. However, further studies with a larger sample size can improve validity of the result.

Objective: Multiple Myeloma (MM) is characterized by uncoupling of bone resorption from bone formation which leads to the predominance of resorption. Bisphosphonates are chemical compounds that selectively concentrate at the interface of the active osteoclasts and the bone resorption surface where they inhibit osteoclast activity. Aim of this study was to describe cytokines behaviour in MM and to evaluate whether zoledronic acid could have an in vivo anti-angiogenic property in MM as observed in solid tumours.
Methods: Serum samples from 29 (16 males and 13 females) consecutive MM patients with lytic bone lesions treated with 4 mg of zoledronic acid were tested for platelet derived growth factor (PDGF), vascular endothelial growth factor (VEGF), interleukin-6 (IL-6), tumor necrosis factor (TNF α) and insulin growth factor (IGF-I). Basal cytokine levels were compared with the values observed after 1, 2, 7 and 21 days of treatment, using the Wilcoxon’s test for nonparametric-dependent continuous variables.
Results: A significant increase in IL-6 and TNF α was observed on days 1 and 2. As for VEGF, the levels of this cytokine did not change significantly from basal values during the entire period of observation except for a significant increase day 7 (P=0.0005). Moreover, PDGF significantly decreased (P=0.005) after 2 days from zoledronic acid infusion.
Conclusions: From this study appears that in MM, treatment with zoledronic acid induces a transient reduction in PDGF according with previous studies in solid cancer, while the increase of IL-6 and VEGF could be related through a paracrine mechanism. The anti-myeloma effect of this drug could be driven through this mechanism.

Introduction: Hemolytic anemia is a common cause of anemia in our country. It may results from a cellular defect that changes the shape of RBCs from biconcave to spherical as in spherocytosis or elliptical as in elliptocytosis. It may results from RBCs enzyme deficiency as in cases with pyruvate kinase deficiency or glucose-6-phosphate dehydrogenase enzyme deficiency (G6PD deficiency). Other causes of hemolytic anemia include hemoglobinopathy‚ autoimmune antibodies against RBCs‚ hypersplenism and others.
G6PD deficiency is the most important disease of the hexose monophosphate pathway and is responsible for two clinical syndromes‚ an episodic hemolytic anemia induced by infections‚ certain drugs or fava beans and a spontaneous chronic non spherocytic hemolytic anemia.
G6PD deficiency is an inherited disorder caused by a genetic defect in the RBCs enzyme G6PD‚which generates NADPH and protects RBCs from oxidative injury. G6PD deficiency is the most common enzymatic disorder of RBCs. Although G6PD is a critical enzyme in the redox metabolism of all aerobic cells‚ yet its role in the RBCs is more critical because it is the only source of NADPH which directly and via GSH defends these cells against oxidative stress. G6PD deficiency is an example of hemolytic anemia due to interaction between an intra-corpuscular cause and an extra corpuscular cause‚ because in the majority of cases hemolysis is triggered by an exogenous agent. The severity of hemolytic anemia varies among individuals with G6PD deficiency. Education of patients and their parents regarding safe and unsafe medications and foods is crucial to prevent future episodes of hemolysis. In many cases all members of the patient’s family should avoid such precipitating foods. G6PD deficiency is a sex-linked disorder. As result males who inherit a G6PD mutation are hemizygous for the defect all their RBCs are affected. Females who inherit a heterozygous G6PD mutation usually do not have severe hemolytic anemia‚ since half of their RBCs express the abnormal allele. The majority of females who inherit an abnormality in G6PD are unaffected carriers. However, the cells that express the abnormal allele are vulnerable to hemolysis as the enzyme deficient RBCs in males. The presence of anemia will vary depending on the severity of the deficiency in the affected cells and whether there is skewed x-inactivation (lyonization) that results in a greater expression in a large percentage of RBCs. G6PD deficiency is a common genetic disorder‚ affecting nearly 400 million individuals worldwide. Whilst it is known that a number of drugs‚ foods and chemicals can trigger hemolysis in G6PD deficient individuals‚ the association between herbal and dietary supplements and hemolysis is less clear. Amongest nutrients blamed as triggers for hemolysis in G6PD deficient individuals are fava beans and related ligaments. Oxidant drugs and infections also predispose to hemolytic attacks. Provided that the blood of a G6P deficient person is normal‚ acute hemolysis results from the action of an exogenous factor on the RBCs deficient in glucose-6-phosphate dedydrogenase (G6PD). The function of G6PD in RBCs is to provide the reduced form of nicotinamide adenine dinucleotide phosphate (NADPH) necessary for the conversion of oxidized glutathione to the reduced state (GSH) as protection against the oxidation of RBCs. So that reduced glutathione (GSH) acts as an anti-oxidant that inactivates oxidant compounds such as hydrogen peroxide‚ that are normally generated within the RBCs. If GSH or any enzyme needed for maintaining glutathione in the reduced form (GSH) is deficient‚ the SH group in the RBCs membrane is oxidized and the hemoglobin of the RBCs is denatured and may precipitate in the RBC inclusions called Heinz bodies. An acute hemolytic process (crisis) results from damage to the RBC membrane by precipitated hemoglobin induced by the exogenous oxidant agent. The damaged RBCs are rapidly removed from the circulation causing acute drop in Hb level and the acute hemolytic crisis. This episodic acute hemolytic anemia may be induced by infections‚ certain drugs and fava beans. Glucose-6-phosphate dedydrogenase (G6PD) deficiency is inherited in an x-linked manner‚ so that the synthesis of RBCs G6PD is determined by a gene on x-chromosome. Thus most females do not usually have evident clinical hemolysis after exposure to oxidant agents‚ unless new gene mutation has occurred. In Egypt ingestion of fava beans produces an acute severe hemolytic crisis known as favism. Fava beans contain divicine‚ isouramil and convicine which ultimately lead to production of hydrogen peroxide and other reactive oxygen species production. Therefore, seasonal increased prevalence of G6PD deficiency crisis occurs in the green beans growth season. This is usually manifested by drop in Hb‚ hemoglobinuria‚ hyperbilirubinemia and reticulocytosis. These patients usually have to be rescued by packed RBCs transfusion. The list of drugs to be avoided in G6PD deficient individuals (whether causing predictable or possible hemolysis) must be given to all cases that present in crisis.
The aim of the study: To find out how much the staff in the Emergency unit are sticking to the agreed upon unit’s protocol as well as to compare the unit’s protocol with European Guidelines.
Subjects: Children from 1 day to 18 years of age with G6PD acute hemolytic crisis attending Assiut University Children Hospital over one year 2015-2016.
Inclusion criteria: All cases of G6PD deficiency acute hemolytic crisis.
Tools of study: The investigations stated in the unit’s protocol included CBCs‚ urine dipsticks‚ blood urea and creatinine‚ serum bilirubin (direct and indirect) as well as blood grouping and cross matching. After treatment of the crisis (as stated in the unit’s protocol) a list of drugs and agents to be avoided was given to the patients. The patients were advised to return to follow up after four weeks for the measurement of G6PD enzyme level in the outpatient department in Assiut university children Hospital.
Patients and methods: This study was performed in the Emergency Unit in Assiut University Children Hospital for one year 2015-2016 (Nov‚ 2015 to Nov‚ 2016). The study included fifty cases of acute G6PD hemolytic crisis admitted to the emergency unit: they were aged nine months to four years. They were 45 males and 5 females. The investigations stated in the unit’s protocol included CBCs‚ urine dipsticks‚ blood urea and creatinine‚ serum bilirubin (direct and indirect) as well as blood grouping and cross matching. After treatment of the crisis (as stated in the unit’s protocol) a list of drugs and agents to be avoided was given to the patients. The patients were advised to return to follow up after four weeks for the measurement of G6PD enzyme level in the outpatient department in Assiut university children Hospital.
Results: The study included fifty cases of acute G6PD hemolytic crisis admitted to the emergency unit: they were aged nine months to four years. They were 45 males and 5 females.

An 81-year-old man presented at hospital with general fatigue, loss of body weight, and high fever. A blood examination showed anemia, thrombocytopenia, neutrophil dysplasia, and the presence of blastoid cells. Bone marrow aspiration also revealed the presence of blast cells and dysmorphic lymphocytes. Flow cytometric analysis of bone marrow cells identified three distinct aberrant cell populations: small and large cells both positive for CD10, CD19 and CD20, and medium-sized to large cells positive for CD13, CD34, and CD117. Cytogenetic analysis of the bone marrow cells showed two distinct populations having either 46, XY, t(8;22)(q24;q11.2), t(14;18)(q32;q21) or 46XY, -5, -17, -20, +mar1, +mar2, +mar3. This is an extremely rare case with concurrence of DHL and AML, and his lymphoma cells and leukemia cells in the bone marrow were difficult to distinguish morphologically. It is essential to fully perform cytogenetic analysis and immunophenotyping for accurate evaluation of bone marrow involvement of DHL.

Cancer disorders are the one of the leading causes of death in most developed and developing countries. They constitute a diverse group of pathologies in which abnormal metabolism and life cycle lead to the profound derangement’s of host metabolism. With the recent advancement in the medical field, many cancer diseases are well controlled with early diagnosis and treatment. So, it is essential that clinicians must be aware the complications of cancer itself and its management. Tumour lysis syndrome (TLS) is an onco-metabolic emergency which occurs due to cancer cells targeted treatment (chemotherapy or radiotherapy) or from rapidly dividing cancer cells itself (spontaneous TLS). TLS was first described by Bedrna and Polcak in 1929 in a chronic leukemia patient treated with radiotherapy. It is characterized by a group of metabolic derangements characterised by hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia and uremia. The consequences are potentially lethal and may result in acute kidney failure, seizures, tetany and even sudden death. The scenario is further complicated in patient with underlying comorbid conditions and in patients at extremes of ages.

The use as the sorbents of activated carbon of different stamps does not give the possibility to extract from the lymph sufficiently the toxic metabolites, biologically active materials, unspecific cytoplasmic ferments, urea, creatinine, bilirubin, amylase, lipase, trypsin, ammonia and other. Furthermore, the existing processes of lymphosorption occupy much time, which is dangerous for the patient, whom necessary to live without the lymph, producing new and expecting purified lymph. The presence in lymph of the oncological patient the tumor cells, which become the sources of metastases in the lymph nodes, where the tumor cells intensely are reproduced because of the stagnancy of lymph and improved the conditions for the reproduction in these places. The necessity of the development of the new technologies of lymphosorption and methods of the destruction of tumor cells in the lymph of oncological patients is appeared. The paper presents the developed fundamentally new process of lymphosorption, which uses action on lymph of electric field and formed under this influence the negatively charged bubbles of hydrogen, and the specially designed everyday medical electroflotators that permit in short time to replace entire lymph of patient into the lymph of healthy person, freed from the cancerous cells, in the continuous regime, by recirculation on the locked outline.
Obtained experimental data permitted to establish the high effective influence of the developed process of lymphosorption on the treatment of patients of liver cancer and hepatitis of all forms. In connection with the fact that lymph is similar on its biochemical characteristics with the plasma and the blood, the developed process and electroflotators can be successfully used for removal from plasma and blood of toxic substances and cancerous cells. It is appropriate to use of the developed method of lymphosorption and the specially designed everyday medical electroflotators for treatment the patients.

The treatment of VTE with direct oral anticoagulant (DOACs) is a common treatment and therapeutic regimens and timing of treatment are well documented by international guidelines both for initial therapy and for extended therapy. Suggested doses of each drug are established after dose-findings studies and thereafter after phase III clinical trials. Suggested doses of edoxaban to treat VTE are based on 60 mg daily while other available dosages, in particular 30 mg daily are suggested if patients show impaired renal function with moderate kidney failure, low body weight or concomitant use of P-glycoprotein inhibitor drugs. However, there are not studies concerning the optimal treatment with any DOACs and in particular for edoxaban for patients aged less than 18 years, because in dose findings studies and in phase III trials alle selected subjects were major than 18 years old; these informations are also lacking in the Literature concerning real life studies too (e.g., case reports and so on). So 30 mg daily of edoxaban for patients younger than 18 years may be considered an off label target. Moreover the off label dosage of 30 mg daily of edoxaban to treat juvenile VTE is related not only to young age but also to other clinical characteristics as juvenile metabolism and body weight and BMI, gastrointestinal absorption and renal and liver metabolism. This report may so add useful information for a next future application of this kind of drugs also in young patients affected by VTE.