Relationship between the proportion of paroxysmal nocturnal hemoglobinuria (PNH) III-erythrocytes and the frequency of exacerbated hemoglobinuria in PNH.
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Complement System Proteins↗
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Paroxysmal cold hemoglobinuria. Transmission and scanning electron microscopy features of erythrocytes.
Djaldetti, Meir, Elion, Dorith, Bessler, Hana, and Fishman, Pnina: Paroxysmal cold hemoglobinuria. Tranmission and scanning electron microscopy features of erythrocytes. Am J Clin Pathol 63. 804-810, 1975. The case history of a patint with paroxysmal cold hemoglobinuria of the chronic iduipathic type is reported. The patient showed a marked dependency on the external temperature, with severe hemolytic anemia and hemoglobinuria during the winter. Examination of the erythrocytes with the transmission electron microscope showed abnormal forms identified as ropalocytes, whereas examination with the scanning electron microscope revealed "holes" in the erythrocytes. (Key words: Paroxysmal cold hemoglobinuria' Ultrastructure of erythrocytes.
Adult↗
Urologic manifestations of paroxysmal nocturnal hemoglobinuria.
A long-term study of 17 patients with paroxysmal nocturnal hemoglobinuria revealed an unexpectedly high incidence of functional and anatomic renal abormalities. All patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. All patients also had granular casts in multiple urinalyses. Evaluation of renal function revealed hyposthenuria, abnormal tubular function and declining creatinine clearance. Radiologically, one or more of these demonstrated enlarged kidneys, renal cortical infarcts and thinning, papillary necrosis, acute renal atrophy, retroperitoneal hematoma and ureteral infarction, which were confirmed by autopsy studies. Hypertension developed in 7 patients. Urinary tract infection was uncommon and no patient had a clinical history compatible with chronic or acute pyelonephritis. Contrary to usual opinion our compatible clearly showed evidence of frequent and widespread renal pathology in paroxysmal nocturnal hemoglobinuria most likely due to repeated microvascular thromboses similar to the venous trombosis involving other organs in this disorder. Since most of these patients present initially to urologists knowledge of this entity is mandatory.
Adolescent↗
Paroxysmal nocturnal hemoglobinuria with onset in childhood and adolescence.
BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematologic disorder characterized by hemoglobinuria, thrombosis, infection, and a tendency toward bone marrow aplasia. Onset usually occurs in adulthood. Few children and adolescents with PNH have been described, and data on diagnosis, clinical course, and survival in young patients are unavailable. METHODS: We retrospectively reviewed clinical and laboratory data on all patients 21 years old or younger in whom PNH had been diagnosed at Duke University Medical Center from 1966 to 1991. RESULTS: Medical records and clinical follow-up data were available for 26 young patients. Although 50 percent of adult patients present with hemoglobinuria, only four of our patients (15 percent) presented with this feature. In contrast, 15 of our patients (58 percent) had moderate or severe bone marrow failure at presentation, as compared with about 25 percent of adults in cases from the literature; all 26 patients eventually had evidence of bone marrow dysfunction. Eight patients (31 percent) have died, with a median survival of 13.5 years since their initial symptoms. CONCLUSIONS: Children and adolescents with PNH have a greater prevalence of bone marrow failure than do adults with this disorder, and their morbidity and mortality are high. Bone marrow transplantation should be considered for selected young patients with PNH.
Adolescent↗
Exertional hemoglobinuria.
A 16 years old boy had a typical exertional hemoglobinuria after walking with his new leather shoes on. The attacks of hemoglobinuria were associated with the appearance of an unstable hemoglobin in red cells. A slightly decreased fragility curve by 24 hr incubated red cells, the increase of autohemolysis and the appearance of heat labile hemoglobin were observed after exertion. These findings support that exertional hemoglobinuria may be regarded as a transitory erythropathia.
Adolescent↗
[Paroxysmal cold hemoglobinuria: only in the textbooks?].
Although it was common in the preantibiotic era, paroxysmal cold hemoglobinuria (PCH) is now an exceptional condition. A 34-year-old female with anemia and episodic hemoglobinuria provoked by cold is reported. The laboratory studies suggested intravascular hemolytic anemia, and the Donath-Landsteiner test showed a biphasic IgG hemolysin with anti-P specificity. This finding and the evidence of latent syphilis were diagnostic of PCH. Penicillin therapy suppressed the hemolytic episodes, although the Donath-Landsteiner test is still positive. It is concluded that PCH is still an exisiting disease which should be considered in the differential diagnosis of episodic hemoglobinuria.
Adult↗
[Postparturient hemoglobinuria in a Quebec dairy herd (author's transl)].
Postparturient hemoglobinuria in a Quebec dairy herdA case of post parturient hemoglobinuria which occurred in a Quebec dairy herd is reported. Anemia and hypophosphoremia were present in many animals without any clinical signs, while others were showing severe signs of hemoglobinuria. History, diagnostic techniques and treatment are discussed.
Animals↗
Platelet hypersensitivity and intravascular coagulation in paroxysmal nocturnal hemoglobinuria.
The patient described had paroxysmal nocturnal hemoglobinuria associated with recurrent arterial as well as venous thrombosis. Study of platelet function revealed hypersensitivity to epinephrine, adenosine 5'phosphate (ADP) and collagen as judged by their ability to aggregate platelets as well as to release 14C serotonin. The release of total nucleotides was also markedly increased over normal with all aggregating agents. The abnormality was localized to the platelet since aggregation occurred when the patient's platelets were resuspended in normal plasma but not when normal platelets were incubated in the patient's plasma. Presumptive evidence for ongoing intravascular coagulation was an increase in fibrinogen derivatives of heavier molecular weight than the native protein presumably a result of thrombin action. However, factor XII was not activated and fibrinolysis was not increased. Complement component levels and antithrombin concentrations were also normal. The findings in this case suggest that hypersensitive platelets may contribute to the intravascular coagulation that is manifested by the increased incidence of thrombosis in patients with paroxysmal nocturnal hemoglobinuria.
Adenosine Diphosphate↗
Renal proximal tubular dysfunction and paroxysmal nocturnal hemoglobinuria.
A patient with paroxysmal nocturnal hemoglobinuria, who required many blood transfusions for hemolytic episodes, had a persistent hyperchloremic metabolic acidosis. Bicarbonate infusion demonstrated a large fractional excretion of bicarbonate (28.6 per cent at a plasma bicarbonate level of 23 meq/liter) which was consistent with proximal renal tubular acidosis. Generalized aminoaciduria and decreased tubular reabsorption of phosphate were also present. Marked deposition of iron in renal proximal tubules was associated with these functional abnormalities. We believe that, as systemic acidosis can promote hemolysis in patients with paroxysmal nocturnal hemoglobinuria, hemolysis can lead, by way of iron deposition in renal tubules, to further acidosis. This cycle should be interrupted with appropriate doses of bicarbonate.
Acidosis, Renal Tubular↗
Renal impairment in patients with paroxysmal nocturnal hemoglobinuria.
Three patients with paroxysmal nocturnal hemoglobinuria accompanied by chronic renal lesions were studied. All the cases had histories of severe hemolytic anemia and repeated hemoglobinuria. The biopsy specimen of the kidney of two patients (Case 1 and Case 2) showed interstitial nephritis. Renal glucosuria, tubular proteinuria, increased urate clearance (Case 2) and reduced tubular reabsorption of phosphate (Case 3) were revealed in Case 2 and Case 3, suggesting renal tubular impairment. From the nephrological point of view, hemodynamic alteration resulting from intravascular hemolysis and severe persistent chronic anemia may primarily be responsible for the renal impairment.
Adult↗
Paroxysmal nocturanal hemoglobinuria and marrow failure treated by infusion of marrow from an identical twin.
An 18-year-old white Canadian male patient with paroxysmal nocturnal hemoglobinuria and refractory marrow failure received a marrow infusion from his normal identical twin brother without prior ablation of the patient's marrow by drugs or irradiation. After 2 years of follow-up, the patient is well with no evidence of clinical disease or significant hematological abnormality. The results suggest that normal marrow stem cells can have a selective advantage over the abnormal paroxysmall nocturnal hemoglobinuria clone.
Adolescent↗
The receptor for urokinase-type plasminogen activator is deficient on peripheral blood leukocytes in patients with paroxysmal nocturnal hemoglobinuria.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal defect in bone marrow-derived cells and is clinically associated with intravascular hemolysis, hemoglobinuria, and an increased frequency of venous thrombosis. The common denominator of PNH-affected blood cells appears to be a defect in the membrane attachment of proteins normally anchored by glycosyl-phosphatidylinositol (GPI). We report here that the cellular receptor for urokinase-type plasminogen activator (u-PAR) is deficient on affected peripheral blood monocytes and granulocytes from four individuals with PNH as evidenced by chemical cross-linking analysis as well as by immunofluorescence flow cytometry using a monoclonal anti-u-PAR antibody. In contrast, on normal blood monocytes and granulocytes we find significant amounts of u-PAR, which is attached to the plasma membrane by a GPI-anchor as defined by its sensitivity towards a specific phospholipase treatment. By two-color flow cytometry it was shown that deficiency of u-PAR expression paralleled that of another GPI-anchored protein. As u-PAR is involved in the initiation of pericellular proteolysis, the reduced expression of u-PAR on PNH-affected leukocytes led to an overall reduction in the capacity for plasminogen activation by cell-surface-bound urokinase. Whereas the abnormal susceptibility of PNH-affected erythrocytes to lysis by autologous complement has been related to the low expression of three GPI-anchored complement regulatory proteins on the cell surface, we now propose that lack of u-PAR expression on the surface of peripheral blood leukocytes may be causally related to the high incidence of venous thrombosis observed in PNH patients.
Animals↗
[Clinical features of paroxysmal nocturnal hemoglobinuria (PNH) in China as compared with those in United Kingdom].
The clinical features of 26 patients of PNH in Sheffield Blood Centre, UK and 50 cases in our hospital, observed in the same period, were compared. Each case was analysed according to the same criteria, and then comparison was made between the two groups. Results showed that in our group: male patients were more common; patients usually had mild or moderate bleeding tendency; the appearance of hemoglobinuria was delayed; abdominal pain was mostly related to hemoglobinuria but not thrombosis; pancytopenia was encountered more frequently and leucopenia as well as thrombocytopenia more remarkable; the incidence of thrombosis was not rare, but occurred rather late: thrombosis was mainly seen in superficial veins, but not veins of viscera; the major cause of death was not thromboembolism but infection. About 25% of the patients achieved long term clinical remission in both groups. None of the patients in these two series transformed to myeloproliferative disorders including acute leukemia. PNH can be considered a benign and chronic hematologic disease.
Adolescent↗
Erythropoiesis in paroxysmal nocturnal hemoglobinuria.
Ten patients with paroxysmal nocturnal hemoglobinuria were studied. The diagnosis was made on the basis of hemolytic anemia, a positive Ham test and hemosiderinuria. Six patients had primary paroxysmal nocturnal hemoglobinuria evolved from aplastic anemia. These four patients also had a milder form of the disease, Over long periods of the follow-up, large variations of hemoglobin values and red blood cell counts were observed. Both absolute and percent reticulocyte counts were increased. Erythroblast counts in the bone marrow were 3-5 times higher than normal. Reticulocyte counts in the bone marrow were 3-5 times higher than normal. Reticulocyte counts showed wide variations but substantially smaller than those in autoimmune hemolytic anemias. Serum iron was either normal or increased, while the bone marrow iron store was high or low. However, the finding of urinary hemosiderin in all cases spoke against depletion of iron stores. The red blood cell life span was moderately shortened. Kinetic studies with 59Fe showed a high red blood cell iron incorporation, while the curves frequently had irregular shapes (broken curve) or an early, abrupt fall. Studies of late erythroid progenitors (CFU-E) indicated that this compartment was preserved. Even after long observation periods was no stem cell pool depletion due to an increased red blood cell demand observed.
Anemia, Aplastic↗
Characteristics of paroxysmal nocturnal hemoglobinuria in China. Clinical analysis of 476 cases.
476 cases of paroxysmal nocturnal hemoglobinuria (PNH) were analysed and compared with PNH patients in foreign countries and its classification was discussed. The clinical characteristics in Chinese PNH patients were: males were affected more; the age of onset was young; more common in north China; hemoglobinuria as an initial symptom was less common; thrombosis as a complication was relatively rare; the common causes of death were heart failure, infections and hemorrhage; and bone marrow picture might show mild dyshematopoiesis.
Adolescent↗
Benign hemoglobinuria following transfusion of accidentally frozen blood.
A patient was transfused with a unit of red blood cells that had been frozen accidentally prior to transfusion. Although he had received approximately 60 gm of free hemoglobin intravenously, the patient's only clinical reaction was massive hemoglobinuria. The benign clinical response, in the presence of massive hemoglobinuria, is attributed to the absence of immunologic imcompatibility and, thus, failure to activate vasoactive mediators and disseminated intravascular coagulation. The case illustrates revised concepts of the pathophysiology of acute renal failure associated with hemolytic blood transfusion reactions.
Accidents↗
Efficacy and Safety of the C3 Inhibitor Pegcetacoplan in Paroxysmal Nocturnal Hemoglobinuria: A Systematic Review and Meta-Analysis.
OBJECTIVE: To evaluate the efficacy and safety of the complement C3 inhibitor pegcetacoplan in patients with paroxysmal nocturnal hemoglobinuria (PNH). METHODS: PubMed, Embase, Web of Science, and Cochrane Library were systematically searched for studies reporting pegcetacoplan use in PNH. Outcomes included transfusion-requirement, hemoglobin normalization, mean hemoglobin levels, lactate dehydrogenase normalization, reticulocyte count normalization, and safety endpoints. Pooled proportions with 95% confidence intervals were calculated using random-effects models, and heterogeneity was assessed using the I2 statistic. RESULTS: Five studies comprising 271 patients were included. Transfusion avoidance was observed in 80.6% of patients, with a pooled transfusion-requirement rate of 19.4%. LDH normalization occurred in 68.5% of patients (I2 = 0%). Hemoglobin normalization was observed in 42.9%, while reticulocyte count normalization reached 66%. Any-grade adverse events occurred in 83.5% of patients, most commonly pyrexia, headache, and dizziness. Serious adverse events occurred in 16.6%, decreasing to 12% after sensitivity analysis. Breakthrough hemolysis was reported in 14.8%, and infections in 17%. CONCLUSION: Pegcetacoplan demonstrates consistent efficacy signals across key hematologic endpoints and an acceptable safety profile, supporting its potential role as an important therapeutic option, particularly in patients with persistent extravascular hemolysis despite C5 inhibition.
Humans↗
Paroxysmal nocturnal hemoglobinuria terminating as erythroleukemia.
A case of a patient who developed erythroleukemia 3 years into the course of paroxysmal nocturnal hemoglobinuria (PNH) is presented. A case of erythroleukemia with a positive sucrose lysis test has been reported, but our case appears to be the first with a long clinical course of PNH evolving into erythroleukemia. The association between these two diseases, their possible clonal origin, and how they fit into the myelodysplastic syndromes are discussed.
Aminosalicylic Acid↗