1/32. Febrile perianal streptococcal dermatitis.We describe a child with an unusual presentation of perianal streptococcal dermatitis which included fever, acral scarletiniform desquamation, and extension of erythema to involve the genitalia and proximal thighs, as well as the commonly seen well-defined erythema of the perianal area. We suggest that isolated group A beta-hemolytic streptococci (GAS) in our patient produced a pyrogenic exotoxin similar to that which appears in scarlet fever.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
2/32. Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia.CONTEXT: patients with sickle cell anemia (SCA) are frequently transfused with red blood cells (RBC). Recently we reported that the calculated risk of RBC alloimmunization per transfussed unit in Brazilian patients with SCA is 1.15%. We describe a delayed hemolytic transfusion reaction (DHTR) presenting as a painful crisis in a patient with SCA. CASE REPORT: A 35-year-old Brazilian female with homozygous SCA was admitted for a program of partial exchange transfusion prior to cholecystectomy. Her blood group was O RhD positive and no atypical RBC alloantibody was detected using the indirect antiglobulin technique. Pre-transfusional hemoglobin (Hb) was 8.7 g/dL and isovolumic partial exchange transfusion was performed using 4 units of ABO compatible packed RBC. Five days after the last transfusion she developed generalized joint pain and fever of 39 degrees C. Her Hb level dropped from 12.0 g/dL to 9.3 g/dL and the unconjugated bilirrubin level rose to 27 mmol/L. She was jaundiced and had hemoglobinuria. Hemoglobin electrophoresis showed 48.7% HbS, 46.6% HbA1, 2.7% HbA2, and 2.0% HbF. The patient's extended RBC phenotype was CDe, K-k , Kp(a-b ), Fy(a-b-), M N s , Le(a b-), Di(a-). An RBC alloantibody with specificity to the Rh system (anti-c, titer 1:16.384) was identified by the indirect antiglobulin test. The Rh phenotype of the RBC used in the last packed RBC transfusion was CcDEe. The patient was discharged, asymptomatic, 7 days after admission.- - - - - - - - - - ranking = 5keywords = hemolytic (Clic here for more details about this article) |
3/32. Three episodes of delayed hemolytic transfusion reactions due to multiple red cell antibodies, anti-Di, anti-Jk and anti-E.There is no report in which three episodes of delayed hemolytic transfusion reaction (DHTR) occurred from multiple antibodies to red cells (RBCs) in the course of treatment of a patient. This paper describes episodes of anemia and hyperbilirubinemia in concert with the development of three alloantibodies in a multiple transfused patient. The patient was a 71-year-old male suffering from valvular heart disease and hemophilia b with a history of transfusions. Although he received compatible RBCs from 14 donors as judged by a crossmatch test using the albumin-antiglobulin method, three episodes of DHTR occurred after surgery. The first hemolytic episode on day 7 after surgery was due to anti-Di(a) because of clinical and laboratory evidence which included jaundice, sudden increases in total bilirubin (T-Bil) and lactate dehydrogenase (LD) levels, and a decrease (2.2 g/dl) in hemoglobin (Hb) level. The second hemolytic episode on day 16 resulted from newly producted anti-Jk(b). The patient experienced fever, fatigue, nausea and anorexia, and laboratory data showed a second increase in T-Bil, a second decrease (3 g/dl) in Hb, and moderate elevations of blood urea nitrogen (BUN) and creatinine (CRE) levels. The third hemolytic episode on day 39 was due to anti-E. The patient complained of fever and fatigue and had a third unexplained drop (1.5 g/dl) in Hb despite no bleeding. This is the first reported case in which three episodes of DHTR occurred from different red cell antibodies.- - - - - - - - - - ranking = 8keywords = hemolytic (Clic here for more details about this article) |
4/32. PFAPA syndrome (Periodic Fever, Aphthous stomatitis, pharyngitis, Adenitis).This paper aims to remind paediatric clinicians to suspect and confirm 'PFAPA' syndrome (Periodic Fever, Aphthous stomatitis, pharyngitis and cervical Adenitis syndrome). We report two cases of PFAPA syndrome: a 3-year-old healthy boy with atopic rhinitis and a boy aged 8 years 5 months who simultaneously had lymphocytic vasculitis syndrome treated with immunosuppressive drugs. Both met Marshall's criteria. The literature regarding PFAPA syndrome was complied using a medline search for articles published between 1963 and 1998 and we then reviewed the reference lists of the articles. The medline search revealed 28 cases with available clinical manifestations, management and prognosis. Our study describes two additional cases. We divided the cases into typical (28 cases) and atypical (two cases) PFAPA syndrome. In typical PFAPA, the age of onset was less than 5 years in most cases and the patients presented 4.9 /- 1.4 days of fever (100%), pharyngitis (89.3%), cervical adenitis (72.1%), stomatitis (71.4%), malaise (64.3%), headache (60.7%), abdominal pain (53.6%) and nausea/vomiting (17.9%). Afebrile intervals were 3.2 /- 2.4 months and increased with age. The time from initial onset to final episode was 3 years 7 months /- 3 years 6 months. The total number of episodes was 8.3 /- 2.5 (range 6-14). Effective treatment included steroids, tonsillectomy/adenoidectomy and cimetidine. The general outcome was good. In atypical PFAPF, the clinical manifestations were similar to those of typical PFAPA except that the age of onset was more than 5 years, and life-threatening intestinal perforation happened once in a patient with underlying Fanconi's anaemia. It was concluded that typical PFAPA syndrome is benign and can be diagnosed by detailed history-taking and from physical findings during repeated febrile episodes with tests to rule out other periodic fever syndromes. A review of the literatures since the first report in 1987 has shown that typical PFAPA syndrome is not associated with significant long-term sequelae and has a good response to steroids. One patient with atypical PFAPA, who received low-dose steroids for over 1 year, developed intestinal perforation after an increment of the 7-day steroid dose. If an underlying problem requires long-term immunosuppressive medication, it is wiser to choose cimetidine rather than increasing the steroid dosage to resolve atypical PFAPA.- - - - - - - - - - ranking = 0.47465433918106keywords = anaemia (Clic here for more details about this article) |
5/32. Malignant pheochromocytoma with cutaneous metastases presenting with hemolytic anemia and pyrexia of unknown origin.We describe a 43 year old female who presented with pyrexia of unknown origin associated with Coomb's negative hemolytic anemia and impaired liver function tests of six months duration. A routine abdominal computerised tomographic scan showed a mass in the left adrenal which was excised at laparotomy and histologically diagnosed as pheochromocytoma. The hemolysis continued to worsen with development of resistance to steroid therapy. Subsequently she developed multiple firm to hard painless cutaneous nodules which were aspirated for cytologic examination. The cytology picture was that of a neuroendocrine tumour with cell morphology similar to that of the adrenal pheochromocytoma. Such an association of hemolytic anemia, pyrexia and cutaneous metastases in a case of malignant pheochromocytoma has not been described earlier in the medical literature.- - - - - - - - - - ranking = 6keywords = hemolytic (Clic here for more details about this article) |
6/32. Cooling mattress induced acute hemolytic anemia.We have reported a patient with Mycoplasma pneumonia, cold agglutinins, and a hemolytic anemia which was probably secondary to vigorous treatment of hyperpyrexia by a cooling mattress. physicians caring for patients with Mycoplasma pneumonia should be alerted to the potential untoward effect of a cooling mattress on an individual with a high titer of cold agglutinins.- - - - - - - - - - ranking = 5keywords = hemolytic (Clic here for more details about this article) |
7/32. dapsone induced cholangitis as a part of dapsone syndrome: a case report.BACKGROUND: dapsone can rarely cause a hypersensitivity reaction called dapsone syndrome, consisting of fever, hepatitis, exfoliative dermatitis, lymphadenopathy and hemolytic anemia. dapsone syndrome is a manifestation of the DRESS (drug rash with eosinophilia and systemic symptoms) syndrome which is a serious condition that has been reported in association with various drugs. cholangitis in dapsone syndrome has not been reported so far in the world literature. CASE PRESENTATION: We report a patient who presented with fever, exfoliative dermatitis, jaundice and anemia within three weeks of starting of dapsone therapy. These features are typical of dapsone syndrome, which is due to dapsone hypersensitivity and is potentially fatal. Unlike previous reports of hepatitic or cholestatic injury in dapsone syndrome we report here a case that had cholangitic liver injury. It responded to corticosteroids. CONCLUSION: We conclude that cholangitis, though unusual, can also form a part of dapsone syndrome. physicians should be aware of this unusual picture of potentially fatal dapsone syndrome.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
8/32. syncope in a middle aged male due to acute rheumatic fever.rheumatic fever is a multi system disease which occurs following infection with group A beta hemolytic streptococcus. It is commonest in the age group of 5-15 years but can occur in adults also. First degree atrioventricular block is a common manifestation of acute rheumatic fever and is included in the Jones criteria but Wenckebacks phenomena and complete heart block are relatively rare manifestations of rheumatic fever. syncope occurring in acute rheumatic fever is also infrequently reported. We report the case of a 38-year-old male with rheumatic carditis who had advanced atrioventricular block which resulted in syncope and required a temporary pacemaker insertion.- - - - - - - - - - ranking = 1keywords = hemolytic (Clic here for more details about this article) |
9/32. Systemic lupus erythematosus presenting as hemolytic uremic syndrome: a case report.Associating systemic lupus erythematosus (SLE), with an initial presentation of hemolytic uremic syndrome (HUS) is rare. We report a case of 21-year old Afghani female admitted to our hospital with an initial complaint of high grade fever and diffuse maculopapular rash and swelling of lower limbs. diagnosis of atypical HUS was established according to the clinical triad of HUS without a veriotoxin-producing organism in her stool and the pathological finding compatible to thrombotic microangiopathy. In addition, her symptoms fulfilled the 1982 revised criteria for the classification of SLE. After pulse methylprednisolone, cyclophosphamide and plasmapheresis therapies, her laboratory findings and general condition improved. Unfortunately she was lost to follow up as she decided to return back to afghanistan.- - - - - - - - - - ranking = 5keywords = hemolytic (Clic here for more details about this article) |
10/32. mycoplasma pneumoniae infection associated with hemolytic anemia--report of one case.Immune hemolytic anemia is a rare condition in childhood. Cold agglutinins have been implicated in the etiology of the hemolysis. However, cold agglutinins may occur in various infections, such as mycoplasma pneumoniae and infectious mononucleosis. In children, especially of preschool age, mycoplasma pneumoniae very commonly cause respiratory tract infection, but inducing an immune hemolytic anemia is exceptional. We present here a case of cold agglutinin-related severe hemolytic anemia (Hb = 3.8 gm/dl) secondary to mycoplasma pneumoniae infection who recovered completely after blood transfusion, intravenous immunoglobulin (IVIG) and medical treatment with erythromycin. It is suggested that even though very rare, mycoplasma pneumoniae may induce the occurrence of cold agglutinins and cause immune hemolytic anemia.- - - - - - - - - - ranking = 8keywords = hemolytic (Clic here for more details about this article) |
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