scholarly journals Acute paroxysmal cold haemoglobinuria; a case report and literature review

2021 ◽  
Vol 20 (3) ◽  
pp. 654-657
Author(s):  
Hasitha Gajaweera ◽  
Kavinda Dayasiri ◽  
Nayani Suraweera ◽  
Chandrachapa Gamage ◽  
Kumudu Weerasekara

Paroxysmal cold hemoglobinuria (PCH) is a very rare subtype of autoimmune hemolytic anemia caused by the presence of cold-reacting autoantibodies in the blood and characterized by the sudden presence of hemoglobinuria, typically after exposure to cold temperatures. The acute onset PCH occurs following viral illnesses whilst the chronic form is secondary to hematological malignancies and tertiary syphilis. It is a complement mediated intravascular hemolytic anemia associated with a biphasic antibody against the P antigen on red cells. We describe a three year child who had acute onset PCH following likely viral infection. The diagnosis was confirmed by demonstration of strongly positive Donnath Landsteiner antibodies. She made a gradual recovery with supportive treatment, ten days following the initial detection of haemolysis. Parents were educated about the need to avoid cold exposure to prevent precipitation of further haemolysis and folic acid was commenced to assist the recovery of erythropoiesis. Bangladesh Journal of Medical Science Vol.20(3) 2021 p.654-657

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Ryan Leibrandt ◽  
Kenneth Angelino ◽  
Monique Vizel-Schwartz ◽  
Ilan Shapira

Paroxysmal cold hemoglobinuria (PCH) is a rare form of cold autoimmune hemolytic anemia first discovered in the early 20th century in adults with tertiary syphilis. Today, it is more commonly seen in children as a life-threatening anemia during a viral upper respiratory tract infection (URI). Although respiratory syncytial virus (RSV) has previously been reported to cause PCH in a child, herein we present the first documented case in an adult. The Donath–Landsteiner (DL) test, the diagnostic test for PCH, was positive on two separate occasions. The patient was treated successfully with warming and avoidance of cold temperatures. To facilitate identification of this rare entity by clinicians, we include a discussion about the pathophysiology, diagnosis, and treatment of PCH.


2021 ◽  
Vol 10 (2) ◽  
pp. 216
Author(s):  
Aikaterini Voulgaridou ◽  
Theodosia A. Kalfa

Autoimmune hemolytic anemia (AIHA) is a rare disease in children, presenting with variable severity. Most commonly, warm-reactive IgG antibodies bind erythrocytes at 37 °C and induce opsonization and phagocytosis mainly by the splenic macrophages, causing warm AIHA (w-AIHA). Post-infectious cold-reactive antibodies can also lead to hemolysis following the patient’s exposure to cold temperatures, causing cold agglutinin syndrome (CAS) due to IgM autoantibodies, or paroxysmal cold hemoglobinuria (PCH) due to atypical IgG autoantibodies which bind their target RBC antigen and fix complement at 4 °C. Cold-reactive antibodies mainly induce intravascular hemolysis after complement activation. Direct antiglobulin test (DAT) is the gold standard for AIHA diagnosis; however, DAT negative results are seen in up to 11% of warm AIHA, highlighting the need to pursue further evaluation in cases with a phenotype compatible with immune-mediated hemolytic anemia despite negative DAT. Prompt supportive care, initiation of treatment with steroids for w-AIHA, and transfusion if necessary for symptomatic or fast-evolving anemia is crucial for a positive outcome. w-AIHA in children is often secondary to underlying immune dysregulation syndromes and thus, screening for such disorders is recommended at presentation, before initiating treatment with immunosuppressants, to determine prognosis and optimize long-term management potentially with novel targeted medications.


2014 ◽  
Vol 25 (1) ◽  
Author(s):  
Hasan M. Isa ◽  
◽  
Lina F. Al Ali ◽  
Afaf M. Mohamed ◽  
Rawia M. Hamad ◽  
...  

1993 ◽  
Vol 52 (3) ◽  
pp. 401-410
Author(s):  
Tsutomu Yamazaki ◽  
Chihiro Harada ◽  
Munetaka Yamakawa ◽  
Tsutomu Sohma

2021 ◽  
Vol 16 (1) ◽  
pp. 746-751
Author(s):  
Tao Wang ◽  
Qiancheng Xu ◽  
Xiaogan Jiang

Abstract A 29-year-old woman presented to the emergency department with the acute onset of palpitations, shortness of breath, and haemoptysis. She reported having an abortion (56 days of pregnancy) 1 week before admission because of hyperthyroidism diagnosis during pregnancy. The first diagnoses considered were cardiomyopathy associated with hyperthyroidism, acute left ventricular failure, and hyperthyroidism crisis. The young woman’s cardiocirculatory system collapsed within several hours. Hence, venoarterial extracorporeal membrane oxygenation (VA ECMO) was performed for this patient. Over the next 3 days after ECMO was established, repeat transthoracic echocardiography showed gradual improvements in biventricular function, and later the patient recovered almost completely. The patient’s blood pressure increased to 230/130 mm Hg when the ECMO catheter was removed, and then the diagnosis of phaeochromocytoma was suspected. Computed tomography showed a left suprarenal tumour. The tumour size was 5.8 cm × 5.7 cm with central necrosis. The vanillylmandelic acid concentration was 63.15 mg/24 h. Post-operation, pathology confirmed phaeochromocytoma. To our knowledge, this is the first case report of a patient with cardiogenic shock induced by phaeochromocytoma crisis mimicking hyperthyroidism which was successfully resuscitated by VA ECMO.


1970 ◽  
Vol 10 (2) ◽  
pp. 133-136 ◽  
Author(s):  
NB Nagaveni ◽  
NB Radhika ◽  
KV Umashankara ◽  
TS Satisha

Transmigration of mandibular canine is an unusual phenomenon characterized by movement of the impacted canine crossing the mandibular midline. Mandibular canine are rarely found impacted in a horizontal position in the mandible. Most of the time, this entity occurred as an isolated finding. However there are reports showing association of dentigerous cyst and a hyperdontia. The purpose of this report is to present a case of transmigrated canine associated with agenesis of mandibular both central incisors which is not reported previously. Key words: Intra-osseous migration; mandibular canine; agenesis; central incisors. DOI: http://dx.doi.org/10.3329/bjms.v10i2.7810 Bangladesh Journal of Medical Science Vol.10 No.2 Apr’11 pp.133-136


2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Demetrio Larraín ◽  
Andrés Casanova ◽  
Iván Rojas

Ovarian torsion after hysterectomy is a rare event. The diagnosis of ovarian torsion is challenging because symptoms are nonspecific. We present a case of ovarian torsion 2 years after laparoscopic hysterectomy (LH). Furthermore, we performed a literature review about ovarian torsion after hysterectomy. This case shows that, in cases of acute onset pelvic pain in patients with history of hysterectomy, the adnexal torsion must be kept in mind in the differential diagnosis, especially in those women who had undergone LH.


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