PNH revisited: Clinical profile, laboratory diagnosis and follow-up

Gupta, Pawan; Charan, VD; Kumar, Harsh

doi:10.4103/0377-4929.44960

Cited by 3 publications

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“…But no PNH clone was detected. This is in contrast with the findings in some studies where cases of PNH were found amongst hypoplastic anemia cases causing pancytopenia [21][22][23][24][25][26].…”

Section: Research Articlecontrasting

confidence: 99%

Diagnostic evaluation of pancytopenia - a prospective institutional study in North-East India

et al. 2016

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Introduction: Pancytopenia, the simultaneous reduction of all the three formed elements of blood, is diverse in its etiology. Full clinico-hematopathological work up is crucial in each case. This study was undertaken with the objectives of identifying the causes of pancytopenia and its phenotypic characteristics in relation to age, sex, ethnicity and socioeconomic status. Methods: Ninety two cases of pancytopenia studied here were diagnosed by complete hemogram study in automated hematology analyzer. Peripheral blood smear studies were carried out. All cases were subjected to bone marrow aspiration/ imprint smear cytology and trephine biopsy study. Hypoplastic anemia cases were investigated for paroxysmal nocturnal hemoglobinuria (PNH) with flow cytometric analysis of red blood cells treated with CD55 and CD59 antibodies. Results: Fifty eight percent of cases were males and mean age was 52 years. Children constituted 16% of patients. Thirty-seven percent of cases belonged to tribal community. Around 70% belonged to low socioeconomic status. Causes of pancytopenia detected were primary hematological malignancies (Total 39.2 % with acute leukaemia 35.9%, lymphoma 2.2% and multiple myeloma 1.1%), hypoplastic anaemia (28.3%), myelodysplastic syndrome (MDS) (21.7%), megaloblastic Anemia (6.5%) and hypersplenism (4.3%). No PNH clone was detected amongst hypoplastic anemia cases. Conclusion: It is concluded that pancytopenia often is the manifestation of serious disease-processes and the commonest cause, in our population of this NorthEastern state of Tripura, being acute leukemia. Bone marrow cytology and biopsy study must be performed in all cases of pancytopenia to find out the cause in order to institute an early treatment.

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Section: Research Articlecontrasting

confidence: 99%

Diagnostic evaluation of pancytopenia - a prospective institutional study in North-East India

et al. 2016

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“…Intravascular hemolysis occurs in PNH because erythrocytes lack the GPI anchor required to attach CD59 to their surface. Extravascular hemolysis also occurs in PNH, due to complement-mediated destruction of erythrocytes by reticuloendothelial macrophages in the liver and spleen [17–19]. In our study we found that the level of CD55 expression on the erythrocytes was significantly lower in β-TM patients compared to healthy controls.…”

Section: Discussionsupporting

confidence: 51%

The effect of splenectomy on complement regulatory proteins in erythrocytes in β-thalassemia major

Kurtoǧllu

Koçtekin

Kurtoğlu

et al. 2019

aoms

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IntroductionHemolysis due to ineffective erythropoiesis is a serious problem β-thalassemia major (β-TM) patients. The role of complement system in the etiopathogenesis of hemolysis observed in β-TM were released. Hemolysis induced by activation of complement system is prevented by complement regulatory proteins. Decay accelerating factor (CD55), membrane inhibitor of reactive lysis (CD59), and complement reception 1 (CR1, CD35) are among these proteins. The absence of these proteins thus accounts for the increased susceptibility of erythrocytes to complement lysis. Splenomegaly and hypersplenism are common complications among thalassemia major patients necessitating splenectomy.Material and methodsIn this study we investigated how splenectomy effects complement regulatory system in erythrocytes. We analysed CD35, CD55, and CD59 levels on erythrocytes in β-TM by flow cytometry.ResultsThe overall mean percentage of CD55 and CD35 positive RBCs of group 1 (22 β-TM with splenectomy) was significantly lower than group 2 (23 β-TM without splenectomy) and group 3 (healthy controls) (p < 0.05). The overall mean percentage CD59 positive RBCs of patients was no significantly different in all groups. The levels of CD35 and CD55 expression on the erythrocytes of splenectomized patients was significantly lower than non-splenectomized patients (p < 0.05).ConclusionsIncreased erythrocyte destruction and iron deposition in organs due to deficiency of these regulatory proteins may be the underlying mechanism of organ damage developing in β-TM patients.

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FLAER Based Assay According to Newer Guidelines Increases Sensitivity of PNH Clone Detection

Manivannan

Tyagi

Pati

et al. 2019

Indian J Hematol Blood Transfus

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PNH revisited: Clinical profile, laboratory diagnosis and follow-up

Cited by 3 publications

References 0 publications

Diagnostic evaluation of pancytopenia - a prospective institutional study in North-East India

Diagnostic evaluation of pancytopenia - a prospective institutional study in North-East India

The effect of splenectomy on complement regulatory proteins in erythrocytes in β-thalassemia major

FLAER Based Assay According to Newer Guidelines Increases Sensitivity of PNH Clone Detection

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