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Background: Polycythaemia is defined as an increase in the hemoglobin above normal. This increase may be real or only apparent because of a decrease in plasma volume (spurious or relative polycythaemia) and the real polycythaemia can be because of primary due to genetic mutations or secondary due to increased erythropoietin production. The dominant symptoms from an increased red cell mass are related to hyper viscosity and thrombosis (both venous and arterial), because the blood viscosity increases logarithmically at hematocrits >55%. The present study aimed to characterize the different clinical presentations and to find out the complications associated with polycythaemia. Aim and objectives: To identify different clinical presentations of polycythaemia and to evaluate various etiologies of polycythaemia. Materials and methods: Patients with polycythaemia presented to OPD or in patient in the Department of General Medicine in Malllareddy Institute of Medical Sciences, Suraram from June 2020 to January 2022 were included. This was a descriptive observational study in which total of 24 patients were analyzed. Patients with polycythaemia (male Hb >16.5g/dl and females Hb>16.0g/dl) were included. Patients admitted with acute dehydration, sepsis and shock and other conditions leading to spurious polycythaemia and patient on diuretics were excluded. All the patients includedwho were found to have increased RBC mass not including patients from exclusion criteria. A detailed clinical history and physical examination was performed to identify clinical patterns of presentation and look for any complications, and necessary lab investigations were sent accordingly, to evaluate for the cause of polycythaemia. Results: A total of 24 patients were included in the study out of which 16 were males .and 8 were females. Polycythaemia Vera was found in 33% while secondary causes were seen in 67%, patients with polycythaemia vera were found to have more thrombotic complications and the mean age of presentation was in between 30- 40 years. Amongst the secondary causes COPD was the most common cause and smoking was the most common risk factor. Conclusion: Polycythaemia presents wide and varied clinical manifestations. polycythaemia rubra vera is a not an uncommon finding in patients presenting with symptoms of arterial or venous thrombosis, polycythaemia rubra vera is more commonly associated with thrombotic tendencies when compared to secondary causes of polycythaemia, when evaluating a case of polycythaemia it is necessary to look for signs and symptoms of secondary causes, to especially look for erythropoietin secreting tumors an high index clinical suspicion is required in such cases.
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Relative polycythaemia is an apparent rise in erythrocyte level in the blood. However, the underlying cause is reduced blood plasma. Relative polycythaemia is often caused by loss of body fluids seen in conditions such as burns, dehydration and stress manifesting itself as a raised Haemoglobin or haematocrit. This case series presents a clinical summary of three patients with acute pancreatitis (AP) and relative polycythaemia due to reduction in plasma volume as a result of intravascular volume depletion as demonstrated by increase in haemoglobin and haematocrit. Haem concentration may be considered as a marker for acute severe pancreatitis and correcting the volume depletion by adequate fluid improves the outcome of AP as seen in all our three patients.
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Awareness and diagnosis of Philadelphia Negative Chronic Myeloproliferative Disorders has now improved and there is a need for more epidemiological data from India. MethodsThis is a retrospective study of patients of polycythaemia conducted at clinical haematology services, BMCRI, Bengaluru from 2010 to 2017. Results88 patients of polycythaemia were retrospectively studied. 84.1% were male and 15.9% were female. Their ages ranged from 19 to 79 years. 75 (85.23%) had Polycythaemia Rubra Vera (PRV). JAK-2 (V617F) mutation was positive in 33.33%. The commonest presentation was with unexplained erythrocytosis in 50 (66.66%), thrombosis in 20 (26.66%) and with bleeding in 2 (2.66%). 22 thrombotic events occurred in 20 PRV patients. Cortical sinus thrombosis was seen in 27.3%, cerebrovascular accidents in 22.8%, portal vein thrombosis in 13.6%, pulmonary embolism in 9.1%, central retinal artery occlusion in 13.6%, myocardial infarction in 4.5% and digital infarction in 9.1% patients. 3 cases of PRV presented with diplopia. No other definitive cause for ocular palsy could be found. The JAK 2 positive group was slightly older than the negative group and had higher frequency of splenomegaly (p<0.05) and higher values for haemoglobin (p<0.001) and neutrophil counts (p<0.001) and platelet counts (p<0.05). ConclusionsPatients with thrombosis, erythrocytosis, thrombocytosis and haemorrhage should be suspected to have myeloproliferative disorders like PRV and investigated. Ophthalmoplegia is a rare presentation and should raise the suspicion for polycythaemia. There is a higher probability of splenomegaly and higher values for haemoglobin and neutrophil counts and platelet counts in JAK 2 positive group.
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The incidence of PAH in CMPD is unknown. When present, PAH in CMPD may be attributed to thrombo–embolism, which is a known complication of CMPD. CMPD and unexplained PAH has been reported in few case reports or few studies with limited cases. Pathophysiology may be related to pulmonary capillary obstruction from increased cellular components, platelet activation and aggregation, micro thrombose formation and stasis. We present the case of a middle aged lady with no previous co–morbidity, who presented with polycythaemia and biventricular failure. Evaluation revealed PAH with no evidence of pulmonary thrombo–embolism. Haematocrit, Serum erythropoietin assay and bone marrow analysis suggested polycythaemia vera. Unexplained PAH in CMPD is discussed.
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Introduction: Microparticles are membrane bound vesicles, measuring less than 1.0 um, which are released during cellular activation or during apoptosis. Studies have shown that these circulating microparticles play a role in coagulation, cell signaling and cellular interactions. Increased levels of circulating microparticles have been observed in a number of conditions where there is vascular dysfunction, thrombosis and inflammation. The objective of this study was to determine the various plasma-derived microparticles in patients with polycythaemia vera (PV) in Universiti Kebangsaan Malaysia Medical Centre and to compare them with normal control. Methods: A total of 15 patients with PV and 15 healthy volunteers were included in this cross-sectional descriptive study. Plasma samples from both patients and healthy volunteers were prepared and further processed for isolation of microparticles. Flow cytometry analyses were then carried out in all samples to determine the cellular origin of the microparticles. Full blood count parameters for both groups were also collected. Data collected were analyzed using SPSS version 12.0. Results: Patients with PV had a significantly higher percentage of platelet derived microparticles compared to healthy controls (P 0.05). Conclusion: The median percentage of positive events for platelet derived microparticles was higher in patients with PV compared to normal healthy controls.
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Hb Tak is one of more than 200 high affinity haemoglobin variants reported worldwide. It resultsfrom the insertion of two nucleotides (AC) at the termination codon, between codon 146 and codon147 of the beta-globin gene [Beta 147 (+AC)]. Polycythaemia is the main clinical feature althoughaffected carriers are usually asymptomatic and do not require intervention. Several case studies inthis region have reported the co-inheritance of Hb Tak with Hb E, delta beta and beta thalassaemiawith one case of homozygous Hb Tak in a Thai boy. In this case report, a cluster of haemoglobinTak was found in a family of Malay ethnic origin. Cascade family screening was conducted whileinvestigating a 4-year old girl who presented with symptomatic polycythaemia. She had 2 previousHb analysis done, at 7-month and 2-year-old with the diagnosis of possible Hb Q Thailand andHomozygous Hb D, respectively. Both diagnosis did not fit her clinical presentations. She was plethoric,had reduced exercise tolerance as well as cardiomyopathy. Her parents were consanguineouslymarried and later diagnosed as asymptomatic carriers of Hb Tak. Consequently, re-analysis of thegirl’s blood sample revealed a homozygous state of Hb Tak. In conclusion, high oxygen affinityhaemoglobin like Hb Tak should be considered in the investigation of polycythaemic patients withabnormal Hb analyses. In this case, DNA analysis was crucial in determining the correct diagnosis.
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Specimens of the west African lungfish, Protopterus annectens (mean weight 294.3 g mean length 38.1 cm), procured from Agulu lake at Agulu in Anambra state, Nigeria were removed from the ambient water and exposed to complete aerial life for a period of seven consecutive days after which some of their haematological changes were determined. There were significant polycythaemia, leucocytosis, thrombocythaemia and elevation of haemoglobin contents (p<0.05). The importance of these haematological adjustments which the fish makes whilst subjected to complete aerial habitat prior to aestivation are discussed.
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20 patients with primitive polycythaemia, aged > 16 were studied at The Central Instutitute of Hematology and Blood Transfusion from August 2000 to July 2001. Patients underwent clinical examinations, specialized test and spinal fluid culture. On 15 of 20 patients, karyotype was specified no abnormality was detected in terms of chromosome number, there are 3 cases of chromosomal morphologic disturbances were considered evidence of hyper trophic characteristics of cloning process us of primitive polycythemia.
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Cromosomas , Policitemia , DiagnósticoRESUMEN
The discovery of an acquired mutation affecting the gene for janus kinase 2(JAK2 V617F)in a high proportion of patients with polycythaemia vera(PV)represents a major breakthrough in molecular understanding of the PV.This article will review the impact of the JAK2 mutation on the diagnosis and treatment of PV.
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That the suggested criteria for diagnosis of polycythaemia vera (PV) are rather complicated, therefore accurate diagnosis of PV from other types of polycythaemia is needed. Serum Vitamin B12 and Vitamin B12 binding proteins were determined in a patients with PV and other 5 patients with secondary polycythaemia. Both serum Vitamin B12 and unsaturated Vitamin B12 binding capacity (UBBC) increased considerably. TCIII and TCI increased also markedly, i.e., 16 and 8 folds, respectively, resulting in a decreased ratio of TCI/TCIII from the normal value of 1.34 to 0.71. Patients with secondary polycythaemia had slightly increased serum Vitamin B12 while serum UBBC, TCI and TCIII levels showed no significant alteration from those of normal subjects. These findings indicated that not only increased serum Vitamin B12 and UBBC but also an increase of TCIII and the ratio of TCIII/TCI are the positive findings in patients with PV. Therefore determinations of serum Vitamin B12 and its binding proteins can be used as a criteria for the differential diagnosis of PV from secondary polythaemia.
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An 80-year-old woman was admitted to the hospital with of pain in the right upper abdominal region. She had splenomegaly and CT scan showed gall stones and splenic infarction. Blood examination revealed erythrocytosis, raised LAP, elevated serum vitamin B12, vitamin B12 binding proteins and reversed TCI/TCIII ratio. All these findings confirmed the diagnosis of polycythaemia vera. After splenectomy and cholecystectomy, a leucoerythroblastic blood picture with teardrop poikilocytosis was detected in the peripheral blood smear. The haemoglobin concentration decreased but leucocytosis and thrombocytosis still persisted. Bone marrow aspirate showed erythroid hypoplasia, decreased megakaryocytes and some degree of fibrosis. This was compatible with the criterial diagnosis of myelofibrosis with myeloid metaplasia. Blood transfusions and myleran were given. Peritoneal dialysis was performed and the patient had severe bleeding and died from cardiac failure due to septic shock. This patient is an example of the transition from polycythaemia vera to myelofibrosis with myeloid metaplasia.