scholarly journals Paroxysmal nocturnal hemoglobinuria associated with infectious mononucleosis

Blood ◽  
1979 ◽  
Vol 54 (2) ◽  
pp. 351-353
Author(s):  
SJ Vogel ◽  
EH Reinhard
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Hemolytic Anemia ◽  
Infectious Mononucleosis ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Reticulocyte Count ◽  
Coombs Test ◽  
Laboratory Evidence ◽  
Cellular Bone ◽  
Symptoms And Signs

A previously healthy 16-yr-old girl was found to have pancytopenia, low reticulocyte count, a cellular bone marrow, and a negative Coombs test, all coincident with clinical and laboratory evidence of infectious mononucleosis. Symptoms and signs of infectious mononucleosis subsided, but pancytopenia and hemolytic anemia persisted. Sucrose hemolysis and acid hemolysis tests supported a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). After 18 mo, the platelet count is normal, but leukopenia and hemolytic anemia continue. The development of PNH in this patient suggests it may have resulted from an effect of infectious mononucleosis.

scholarly journals Paroxysmal nocturnal hemoglobinuria associated with infectious mononucleosis

Blood ◽  
1979 ◽  
Vol 54 (2) ◽  
pp. 351-353 ◽  
Author(s):  
SJ Vogel ◽  
EH Reinhard
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Hemolytic Anemia ◽  
Infectious Mononucleosis ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Reticulocyte Count ◽  
Coombs Test ◽  
Laboratory Evidence ◽  
Cellular Bone ◽  
Symptoms And Signs

Abstract A previously healthy 16-yr-old girl was found to have pancytopenia, low reticulocyte count, a cellular bone marrow, and a negative Coombs test, all coincident with clinical and laboratory evidence of infectious mononucleosis. Symptoms and signs of infectious mononucleosis subsided, but pancytopenia and hemolytic anemia persisted. Sucrose hemolysis and acid hemolysis tests supported a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH). After 18 mo, the platelet count is normal, but leukopenia and hemolytic anemia continue. The development of PNH in this patient suggests it may have resulted from an effect of infectious mononucleosis.

The Immature Platelet Fraction in HIV Patients with Thrombocytopenia.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2095-2095
Author(s):  
Brian T. Garibaldi ◽  
Rupal B. Malani ◽  
Hsin-Chieh Yeh ◽  
Deborah Michell ◽  
Evan J. Lipson ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Bone Marrow ◽  
Platelet Count ◽  
Peripheral Blood ◽  
Reticulocyte Count ◽  
Control Group ◽  
P Value ◽  
Hiv Patients ◽  
Immature Platelet Fraction ◽  
The Mean

Abstract Thrombocytopenia is a common clinical feature of HIV infection. Given the number of possible etiologies of thrombocytopenia in a patient with known HIV, a peripheral blood test effective in determining the likely pathophysiologic basis of the thrombocytopenia would be a valuable clinical tool. Immature platelets are released early from the bone marrow in response to increased platelet turnover. These platelets contain residual megakaryocyte mRNA and have been termed reticulated platelets. A new assay, the Immature Platelet Fraction (IPF), measures the reticulated platelet count in peripheral blood. Patients with increased destruction of platelets from such conditions as ITP consistently have a higher IPF percent, while patients with decreased platelet production have a low or normal IPF percent. The goal of our study was to determine the performance characteristics of the IPF assay in HIV patients with thrombocytopenia and to see if the IPF percent could be used to help elucidate the etiology of low platelet counts in this group of patients. All adult patients admitted to the Johns Hopkins Hospital with a diagnosis of HIV and a platelet count less than 150,000 were eligible for enrollment. 62 patients were identified from February 2007 to June 2007. 34 control samples were obtained from inpatients with HIV who were not thrombocytopenic. In addition, 81 samples were available from non-HIV historical controls with normal platelet counts. The mean platelet count in the HIV thrombocytopenic group was 92,000 while the mean platelet count in the HIV control group was 254,000 (p value <.001). The mean platelet count in the non-HIV historical control group was 274 (p=.34 when compared to the HIV control group). The mean IPF percent in the HIV thrombocytopenic group was 10.2% as compared to 6.8% in the HIV control group (p=.001). The mean IPF in historical non-HIV controls was 3.1% (p<.001 for both the HIV thrombocytopenic and the HIV control group). Univariate analyses were conducted to identify potential individual predictors of a high IPF percent. Backward selection was then performed using multivariate linear models with a threshold Wald test p-value of 0.05. ITP, diabetes mellitus and cirrhosis were significantly associated with a higher IPF percent with a co-efficient (95% confidence interval) of 6.98 (3.05–10.91), 4.73 (1.39–8.06), and 14.18 (9.7–18.66), respectively. CD4 count, HIV viral load, hepatitis C and reticulocyte count were not correlated with IPF percent. Our results suggest that patients with HIV have increased platelet turnover as compared to patients without HIV. Thrombocytopenic patients with HIV have increased platelet turnover relative to both non-thrombocytopenic HIV patients and to historical non-HIV controls. History of ITP, diabetes mellitus, and cirrhosis are predictive of an elevated IPF percent. Reticulocyte count is not correlated to IPF percent, suggesting that a low reticulocyte count is not a reliable marker for decreased bone marrow production in HIV thrombocytopenia. It is unlikely that the IPF assay alone can be used to determine the pathophysiologic basis of thrombocytopenia in any single patient with HIV. Further work needs to be done to clarify the utility of the IPF assay in this group of patients.

scholarly journals Autoimmune myelofibrosis associated with autoimmune hemolytic anemia successfully treated with ruxolitinib

2021 ◽  
Author(s):  
Shuku Sato ◽  
Wataru Kamata ◽  
Yotaro Tamai
Keyword(s):  
Bone Marrow ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Transforming Growth Factor ◽  
Myeloproliferative Neoplasms ◽  
Bone Marrow Examination ◽  
Coombs Test ◽  
Bone Marrow Fibrosis ◽  
Cytokine Levels ◽  
Antiglobulin Test

Abstract A 55-year-old man suffered from dyspnea, general malaise, and jaundice. His laboratory date showed pancytopenia and hemolytic anemia, and computed tomography showed splenomegaly. Bone marrow examination revealed myelofibrosis (MF)-1. The hemolytic anemia was diagnosed as IgM autoimmune hemolytic anemia (AIHA) with negative direct and indirect Coombs test but positive IgM-direct antiglobulin test. We started ruxolitinib 20 mg, which improved not only bone marrow fibrosis, symptoms related to myeloproliferative neoplasms and splenomegaly, but also AIHA. AIHA may be associated with Autoimmune MF (AIMF), and cytokines such as transforming growth factor (TGF)-β are thought to be involved in such cases. This case suggests that ruxolitinib may improve the cytokine levels and may lead to the treatment of AIHA as well as AIMF.

Pregnancy Associated Thrombotic Thrombocytopenic Purpura: A Case Report.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3221-3221
Author(s):  
Xu Ye ◽  
Ying Feng ◽  
Ying Pang ◽  
Zhaoyue Wang
Keyword(s):  
Bone Marrow ◽  
Renal Function ◽  
Platelet Count ◽  
Case Report ◽  
Partial Response ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Hemolytic Anemia ◽  
Oral Prednisolone ◽  
Thrombocytopenic Purpura ◽  
Blood Routine

Abstract Introduction: In clinical setting, cases with thrombotic thrombocytopenic purpura (TTP) are easily mis-diagnosed and the clinical outcome may be influenced by the mis-diagnosis. Case report: The patient is a 28-year-old woman in her first pregnancy. She had transient thrombocytopenia two years ago and was diagnosed idiopathic thrombocytopenic purpura (ITP) based on her bone marrow results but recovered quickly without immunosuppressive therapy. Her blood routine test was normal before the pregnancy. But she was found to have asymptomatic thrombocytopenia during her first pregnancy check-up in the first trimester of her pregnancy. A diagnosis of ITP was made after bone marrow examination. Then, she was put on a course of oral prednisolone and her platelet count transiently increased to normal but decreased to 18×109/L after she cut down on the dose. After that, she only showed partial response to increased dose of oral prednisolone and her hemoglobin (Hb) level began to fall slowly. She was admitted during her second trimester of gestation complaining of headache and low fever with signs of agitation. Blood routine, blood smear analysis, urine routine, liver function and blood bilirubin analysis, renal function tests were performed regularly. Bone marrow smear examination, tests for detecting various causes of hemolysis and tests detecting autoimmune antibodies were also performed. Her lab tests were indicative of severe hemolytic anemia and thrombocytopenia. Her renal function was continuously normal. As other causes of hemolysis and autoimmune diseases were excluded, a diagnosis of Evan’s syndrome was made although the Coombs’ test was negative. She showed partial response to high-dosage of prednisolone and IgG with increased but not normal platelet count and her fever disappeared. After transfusion of packed RBC and platelets, her symptoms and signs of the central nervous system disappeared and the fetus was removed through cesarean section. 22 days later, she underwent an emergent exploratory laparotomy confirming acute necrotic cholecystitis under supportive transfusion, and splenectomy was also performed. But the hemolytic anemia and thrombocytopenia still went on although she was still administered prednisolone and high-dosage of IgG. As a diagnosis of TTP was suspected, she received plasma exchange. The next day, her Hb level increase to 97g/L and her platelet count increased to 500×109/L without transfusion. More than one month later, her plasma sample was sent to another center to detect ADAMTS13 level and was found to be deficient in ADAMTS13 (7%). But antibody to ADAMTS13 was not detected. Conclusion: The cause of anemia and thrombocytopenia in this case was pregnancy associated TTP. Further experiment for detection of mutation in her ADAMTS13 gene is being done. Mis-diagnosis in this case may be related to lack of recognition of variation in the course, precipitating factors of the disease and unavailability of ADAMTS13 level test.

High-Sensitivity Flow Cytometry Testing for Paroxysmal Nocturnal Hemoglobinuria in Children with Cytopenia: A Single Center Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2398-2398
Author(s):  
Choladda V. Curry ◽  
M. Tarek Elghetany ◽  
Andrea M. Sheehan ◽  
Alison A. Bertuch ◽  
Ghadir S. Sasa
Keyword(s):  
Bone Marrow ◽  
Flow Cytometry ◽  
Aplastic Anemia ◽  
Immunosuppressive Therapy ◽  
Hemolytic Anemia ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
High Sensitivity ◽  
Deficiency Anemia ◽  
Unknown Etiology ◽  
Flow Cytometry Assay

Abstract Abstract 2398 Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disorder characterized by expansion of cells with complete or partial loss of glycosyl phosphatidyl-inositol-anchored proteins. PNH usually presents with one or more of three clinical manifestations: intravascular hemolysis, thrombosis, or acquired bone marrow failure [aplastic anemia (AA) or myelodysplastic syndrome (MDS)]. Flow cytometry has become the gold standard for the diagnosis of PNH, particularly with the recent publication of guidelines for the diagnosis and monitoring of PNH and related disorders in 2010. PNH occurs rarely in children, and, consequently, the published literature regarding PNH in this pediatric population consists only of small case series, making it difficult to extrapolate the frequency of which PNH clones are identified. Moreover, no studies are available on the incidence of PNH clones in children with MDS and acquired aplastic anemia (AAA). We, therefore, sought to determine how frequently a high sensitivity FLAER-based assay, with a sensitivity of 0.01%, would detect PNH clones in children with cytopenias. Method and Results: The study period was from December 2010 to July 2011. PNH testing was performed using a high sensitivity FLAER based assay according to published guidelines using the combination of FLAER/CD64/CD15/CD33/CD24/CD14/CD45 for WBC testing and CD235a/CD59 for RBC testing. There were 31 peripheral blood samples from 29 patients (17 males/12 females) ranging in age from 4 months to 17 years (median, 10 years). All patients were tested for PNH because of cytopenia [pancytopenia (n = 14) and uni- or bicytopenia (n = 15)]. Patients had a mean Hgb of 10.7 gm/dL, mean ANC of 2.66 X103/uL and mean platelet of 115 X103/uL. Review of medical charts revealed the following clinical diagnoses: classic PNH - episodic hemolytic anemia with persistent thrombocytopenia (1), severe AA (SAA, 8), SAA with myelofibrosis (1), MDS (1), Fanconi anemia (1), chronic thrombocytopenia (2), refractory iron deficiency anemia (1), bone marrow suppression likely due to virus/medication (1), parvovirus infection (1), Copper deficiency (1), systemic lupus erythematosus (SLE, 1), and cytopenia of unknown etiology (10). Of note, all patients with AAA had SAA. PNH clones were identified in 6 out of 29 patients (20%): minor clones (<1% PNH population) in 3 patients: average clone sizes 0.12% [range 0.02–0.25] granulocytes (G), 0.51% [0.20–0.99] monocytes (M), and 0.08% [0.04–0.14] red blood cells (RBCs), and major clones (>1% PNH population) in 3 patients: average clone sizes 31.11% [3.98–67.58] G, 31.98% [6.15–71.1] M, and 14.76% [1.19–38.03] RBC, respectively, with ages ranging from 4 to 17 years. Patients who were identified to have minor PNH clones all presented with pancytopenia. Two were diagnosed with SAA; the cause of pancytopenia in the third patient is currently under investigation. None of patients with minor PNH clones had evidence of hemolysis or thrombosis. The three patients with major PNH clones had the following: Classic PNH with hemolytic anemia (1), SAA with PNH clones detected at the time of SAA diagnosis (1), and SAA with PNH clones detected 20 months after immunosuppressive therapy (1). The latter two patients did not have evidence of hemolysis or thrombosis. Of the 10 patients with a diagnosis of SAA or MDS, PNH clones were identified in 4 (40%) patients (2 with minor clones, 2 with major clones). Conclusions: This is the first study to describe the utility of using a standardized high-sensitivity FLAER-based flow cytometry assay to identify PNH clones in children. This is also the first study describing the prevalence of PNH clones in children with MDS and AAA. The identification of a PNH population in 40% of the MDS and AAA cases emphasizes the need for PNH testing in all children with these disorders using a high-sensitivity FLAER based flow cytometry assay. A low sensitivity assay would have missed 2 patients with minor PNH clones. This finding may be of significance considering SAA or MDS patients with PNH clones are more likely to respond to immunosuppressive therapy. Further studies are needed to investigate the prevalence of PNH clones in this setting and its impact on disease manifestations, course, and outcomes in children. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Case Report: Possible Paroxysmal Nocturnal Hemoglobinuria with Pronounced Pancytopenia, Reticulocytopenia, and without Hemoglobinuria Simulating Aplastic Anemia

Blood ◽  
1952 ◽  
Vol 7 (8) ◽  
pp. 842-849 ◽  
Author(s):  
HENNING LETMAN
Keyword(s):  
Bone Marrow ◽  
Case Report ◽  
Aplastic Anemia ◽  
Hemolytic Anemia ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Serum Bilirubin ◽  
Refractory Anemia ◽  
Severe Anemia ◽  
True Nature ◽  
Congenital Hemolytic Anemia

Abstract 1. This paper describes a case of chronic severe anemia with very pronounced neutropenia and thrombocytopenia and a varying degree of reticulocytopenia. Crosby’s modification of the Ham test for paroxysmal nocturnal hemoglobinuria proved to be strongly positive. Despite a prolonged period of observation no unquestionable hemoglobinuria was found, but slight hemoglobinemia was found. As the serum bilirubin was normal and there was no reticulocytosis an ordinary hematologic examiation could not have revealed the hemolytic nature of the anemia. Because of the pronounced pancytopenia and the lack of response to all therapy, the case would presumably have been classified as one of aplastic or "refractory" anemia. 2. The supposition suggests itself that other cases of aplastic or "refractory" anemia and of hemolytic anemia without hemoglobinuria are actually atypical forms of paroxysmal nocturnal hemoglobinuria. The hemoglobinuria in this disease is an immaterial symptom. It should be possible to reveal such atypical cases by means of Ham’s and Crosby’s tests. 3. Pancytopenia is seen not only in the so-called hypersplenic hemolytic anemia, in which the bone marrow is hyperplastic but in congenital hemolytic anemia during crisis and in certain cases of auto-immune acquired hemolytic anemia. It is therefore possible that other forms of hemolytic anemia may be cloaked by a picture of "aplastic anemia." 4. From the standpoint of therapy, it is naturally important to settle the question as to the true nature of the anemia. In congenital and in hypersplenic hemolytic anemia, splenectomy exerts a curative effective; in acquired hemolytic anemia caused by antibodies, treatment with ACTH and cortisone will be effective in many cases; and, finally, in PNH no therapy other than transfusions is presently available.

scholarly journals An Interesting Presentation of Severe Bicytopenia in A Case of SLE

2021 ◽  
Vol 8 (1) ◽  
pp. C17-19
Author(s):  
M Aswin Manikandan ◽  
A Josephine ◽  
Vindu Srivastava ◽  
S Mary Lilly
Keyword(s):  
Platelet Count ◽  
Haemolytic Anaemia ◽  
Blood Count ◽  
Complete Blood Count ◽  
Reticulocyte Count ◽  
Coombs Test ◽  
Peripheral Smear ◽  
History Of ◽  
Wbc Count ◽  
Transfusion Reactions

We present a case of 25-year-old female who was brought to the hospital for complaints of generalised weakness, fever, and history of melena and haematuria. Following admission complete blood count and peripheral smear was asked; Complete blood count (CBC) findings were haemoglobin 4.2 gm/dl, total WBC count was 14,990, platelet count 7000, reticulocyte count 4%, NRBCs were 15/100 WBCs. Peripheral Smear showed fragmented RBCs, polychromatophils microspherocytes and multiple foci of autoagglutination suggestive of autoimmune haemolytic anaemia. Pertaining to these findings immune workup was done for this patient; coombs test was negative for this patient, but ANA was positive. This identification proved valuable to the patient as; administration of corticosteroids helped to prevent haemolytic transfusion reactions and improved the patient’s haemoglobin and platelet count.

scholarly journals Paroxysmal Nocturnal Hemoglobinuria

2005 ◽  
Vol 44 (157) ◽  
Author(s):  
Buddhi P Paudyal ◽  
M Zimmerman ◽  
A Karki ◽  
H Neupane ◽  
G Kayastha
Keyword(s):  
Bone Marrow ◽  
Hemolytic Anemia ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Bone Marrow Failure ◽  
Chronic Hemolysis ◽  
Tremendous Impact

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hemolytic disorder of acquired origin and is clinically manifested by chronic hemolysis, thromboses in various sites, and bone marrow failure. The disease isso rare that the delay in the diagnosis is not uncommon and this bears a tremendous impact on patientmanagement. We present this case to draw attention to this uncommon cause of hemolytic anemia, whichshould be considered in any patient, of any age, who has signs of chronic hemolysis.Key Words: Paroxysmal Nocturnal Hemoglobinuria, Hemolytic anemia.

Novel Anti-Hemoglobin F Antibody in Bone Marrow Can Predict a Response to Antithymocyte Globulin and Cyclosporine in Patients with Aplastic Anemia. Retrospective Study of 28 Cases.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1059-1059
Author(s):  
Ryoichi Ichihashi ◽  
Masafumi Ito ◽  
Naoyoshi Mori ◽  
Seiji Kojima
Keyword(s):  
Bone Marrow ◽  
Platelet Count ◽  
Neutrophil Count ◽  
Antithymocyte Globulin ◽  
Reticulocyte Count ◽  
Complete Response ◽  
Hemoglobin Level ◽  
University Hospital ◽  
Red Cross ◽  
Hemoglobin F

Abstract The distinction between hypoplastic myelodysplastic syndrome (MDS) from AA by morphology alone is sometimes difficult. It is required to invent a new tool to differentiate two diseases. We developed a novel polyclonal antibody by immunizing a rabbit with synthetic peptide of human HbF, which can detect of HbF-containing erythroblasts in paraffin-embedded hematpoietic tissues. HbF is a useful marker to distinguish MDS from AA. Although 42 of 51 MDS patients with cytogenetic abnormalities were positive with HbF, only 2 of 12 patients with typical AA were positive in previous study. (Ichihashi R, et al: Blood102: 428a, 2003). On the basis of previous findings, we hypothesized that HbF expression on bone marrow erythroblast might be applied to predict to IST in AA patients. Material and methods: We investigated bone marrow samples from 24 patients with acquired AA who received IST with antithymocyte globulin (ATG) and cyclospoline (CSA) between 1993 and 2004 in Nagoya University Hospital and Japanese Red Cross Nagoya First Hospital. The diagnosis of AA was established by bone marrow findings and peripheral blood counts. As for grading, the disease was considered severe if at least two of the following were noted: a neutrophil count of less than 0.5×109/L, a platelet count of less than 20×109/L, a reticulocyte count of less than 20×109/L with hypocellular bone marrow. Moderate disease was defined by at least 2 of the following hematologic values: a neutrophil count of less than 1.0×10×109/L, a platelet count of less than 50×109/L and reticulocyte count of less than 60×109/L. 23 Patients had severe AA and 5 had moderate AA. Patient’s age ranged from 2 to 80 years (median: 25.7 years), Complete response (CR) was defined as a neutrophil count > 1.5×109/L, a platelet count > 100×109/L, and a hemoglobin level of > 11.0g/dl. Partial response (PR) was defined as a neutrophil count > 1.0×109/L, a platelet count > 30×109/L, and a hemoglobin level of > 8.0g/dl in patients with sever AA. It was defined as a neutrophil count > 1.0×109/L, a platelet count >3.0×109/L in patients with moderate AA. Formalin fixed, paraffin embedded bone marrow tissues were immunostained. We defined that HbF was positive when more than 3 erythroblasts were stained in a cluster. Results: Overall response rate was a 46.4percentage of patients with CR and PR at 6 months after IST. Higher probability of response to IST was observed in patients with HbF-negative AA. Although 5 of 7 patient (71.4%) with HbF-negative AA responed to ISF, only 8 of 21 patient (38.1%) with HbF-positive AA responded. Of 15 patients who did not respond to IST, HbF positive erythreblasts were detected in 13 patients (86.7%). Conclusion: These results suggest that staining of HbF positive erythroblast in pretreatment bone marrow samples may be helpful to predict a good response to IST especially in adults. HbF-positive AA may have common clinical and biological basis with MDS. We are planning to use this novel tool to distinguish AA from MDS prospectively. HbF and Response to ATG, Adults HbF+ HbF− *All 5 cases: Not examined CR/PR 3 (3cases:DR2+) 3 (3cases:DR2+) NR/BMT 5(DR2+*) 0 HbF and Response to ATG, Children HbF+ HbF− CR/PR 5 (3cases :DR2+) 2 NR/BMT 8 (3case: DR2+) 2 (1case:DR2+)

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