scholarly journals Myelodysplasia in the setting of paroxysmal nocturnal hemoglobinuria: Interpretation of blast percentage in a marrow with erythroid hyperplasia

eJHaem ◽  
2020 ◽  
Vol 1 (2) ◽  
pp. 404-405
Author(s):  
Emma M. Groarke ◽  
Bhavisha A. Patel ◽  
Alina Dulau‐Florea ◽  
Irina Maric ◽  
Neal S. Young ◽  
...  
Keyword(s):  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Erythroid Hyperplasia

scholarly journals Paroxysmal Nocturnal Hemoglobinuria: Diagnostic Challenges in Pediatric Patient

2019 ◽  
Vol 2019 ◽  
pp. 1-5 ◽  
Author(s):  
Dharshana Krishnaprasadh ◽  
Inna Kaminecki ◽  
Anna Sechser Perl ◽  
Jonathan Teitelbaum
Keyword(s):  
Bone Marrow ◽  
Small Bowel ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Bone Marrow Failure ◽  
Exploratory Laparotomy ◽  
Recurrent Abdominal Pain ◽  
Histological Evaluation ◽  
Small Vessel Vasculitis ◽  
Wall Thickening ◽  
Erythroid Hyperplasia

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, life-threatening hematologic stem cell disorder characterized by hemoglobinuria, thrombosis, and tendency for bone marrow failure. The rare incidence of PNH in children, its nonspecific clinical presentation, and occasional absence of hemoglobinuria make the diagnosis challenging. We present a case of a 17-year-old boy who was hospitalized with a history of recurrent abdominal pain, fever, and dark-colored urine. Laboratory tests revealed anemia, thrombocytopenia, and elevated inflammatory markers. Urinalysis was positive for protein and red blood cells, too many to be counted. Complement studies were within normal limits. Abdominal computed tomography showed a segment of the small bowel with wall thickening and signs of possible microperforation. Exploratory laparotomy revealed necrosis of the small bowel, and histological evaluation was suggestive of an autoimmune process with small vessel vasculitis. Bone marrow biopsy showed hypocellular marrow with a decreased number of myeloid cells, normal number of megakaryocytes, and signs of erythroid hyperplasia. Flow cytometry detected deficiency of CD59 leading to the diagnosis of PNH. The patient was treated with eculizumab infusions resulting in significant improvement. This case highlights the need for high clinical suspicion for rare entities such as PNH in patients presenting without hemoglobinuria.

RBC transfusions in paroxysmal nocturnal hemoglobinuria

1977 ◽  
Vol 137 (4) ◽  
pp. 536-538 ◽  
Author(s):  
J. P. Gockerman
Keyword(s):  
Paroxysmal Nocturnal Hemoglobinuria

Radiation Sickness after Therapeutic Administration of 806 mCi of 131I for Thyroid Cancer

1967 ◽  
Vol 06 (02) ◽  
pp. 170-183
Author(s):  
K. Šilink ◽  
J. Němec ◽  
J. Kubal ◽  
S. Röhling ◽  
S. Vohnout
Keyword(s):  
Bone Marrow ◽  
Thyroid Cancer ◽  
Radiation Injury ◽  
Clinical Course ◽  
External Irradiation ◽  
Bone Marrow Depression ◽  
Erythroid Hyperplasia ◽  
Cytological Features ◽  
Haematological Changes ◽  
Therapeutic Administration

SummaryThe clinical course and the haematologic events in a patient suffering from metastatic thyroid cancer after administration of 806 mCi of 131I are described. A serious bone marrow depression developed and was treated successfully. The haematological changes during the early and late phases of the radiation injury were studied in detail and compared with those after external irradiation. The haematological events after internal irradiation with 131I are characterised by initial neutrophilic leukocytosis, protracted lymphopenia, slowly developing anaemia reaching lowest values about 3 months after administration, erythroid hyperplasia in the bone marrow after recovery from bone marrow depression and prominent cytological features of the bone marrow, especially pronounced erythropoietic polyploidy.

Vitamin B12 Deficiency an Unusual Cause of Fever: A Case Report

2019 ◽  
Vol 4 (02) ◽  
Author(s):  
Shyama . ◽  
P. Kumar ◽  
Surabhi .
Keyword(s):  
Bone Marrow ◽  
Vitamin B12 ◽  
Unusual Case ◽  
Vitamin B12 Deficiency ◽  
Bone Marrow Aspiration ◽  
Microcytic Anemia ◽  
Peripheral Smear ◽  
Erythroid Hyperplasia ◽  
B12 Deficiency ◽  
Vitamin B12 Supplementation

Introduction: An unusual case of a 19 year old female, presenting with fever, pallor and hepatosplenomegaly for one month. She had microcytic anemia on peripheral smear examination but her bone marrow aspiration & biopsy revealed a hypercelluar marrow with megaloblastic erythroid hyperplasia. Resolution of fever within 48 hours of Vitamin B12 supplementation, initiated in view of the megaloblastic bone marrow picture & low serumVitamin B12 level, suggests a causal association. Conclusion: Vitamin B12 deficiency seems to be an unusual cause of PUO (Pyrexia of unkown origin) which should be ruled out in every case of PUO.

scholarly journals SUPPRESSION OF MYELOPOIESIS IN THE SOFT AGAR CULTURE SYSTEM IN A PATIENT WITH PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH) AND APLASTIC ANEMIA (AA)

1977 ◽  
Vol 11 (4) ◽  
pp. 492-492
Author(s):  
Norma K C Ramsay ◽  
William Krivit ◽  
Mark E Nesbit ◽  
Peter F Coccia ◽  
John H Kersey
Keyword(s):  
Aplastic Anemia ◽  
Culture System ◽  
Paroxysmal Nocturnal Hemoglobinuria ◽  
Soft Agar ◽  
Agar Culture

scholarly journals Successful discontinuation of anticoagulation following eculizumab administration in paroxysmal nocturnal hemoglobinuria

2009 ◽  
Vol 84 (10) ◽  
pp. 699-701 ◽  
Author(s):  
Ashkan Emadi ◽  
Robert A. Brodsky
Keyword(s):  
Paroxysmal Nocturnal Hemoglobinuria

Paroxysmal nocturnal hemoglobinuria complicated with essential thrombocythemia harboring concomitant PIGA, CALR, and ASXL1 mutations

2021 ◽  
Author(s):  
Haruya Okamoto ◽  
Nobuhiko Uoshima ◽  
Yuri Kamitsuji ◽  
Eri Kawata ◽  
Yukiko Komori ◽  
...  
Keyword(s):  
Essential Thrombocythemia ◽  
Paroxysmal Nocturnal Hemoglobinuria
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