A 22-year old female patient with systemic lupus erythematosus presenting microangiopathic hemolytic anemia was treated with therapeutic plasma exchange 23 times. The patient's condition and laboratory findings (aspartate aminotransferase, alanine aminotransferase, ferritin, total bilirubin, and lactate dehydrogenase) did not improve despite the initial 18 therapeutic plasma exchange treatments. Thrombotic thrombocytopenic purpura was ruled out due to normal ADAMTS-13 activity test result; hemophagocytic lymphohistiocytosis was diagnosed based on fever, splenomegaly, pancytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow aspiration. The patient's condition improved rapidly upon treatment with a combination of immunosuppressants and cytotoxic agents, and more therapeutic plasma exchanges were performed five consecutive times with prolonged intervals in between. We observed that therapeutic plasma exchange treatment alone was not effective enough to treat hemophagocytic lymphohistiocytosis, unlike thrombotic thrombocytopenic purpura. Therefore, it is necessary to determine and start drug administration promptly in the treatment of hemophagocytic lymphohistiocytosis with thrombotic microangiopathy.
Alanine Transaminase ; Anemia, Hemolytic ; Bilirubin ; Bone Marrow ; Cytotoxins ; Female ; Ferritins ; Fever ; Humans ; Hypertriglyceridemia ; Immunosuppressive Agents ; Lactic Acid ; Lupus Erythematosus, Systemic ; Lymphohistiocytosis, Hemophagocytic ; Pancytopenia ; Plasma Exchange ; Plasma ; Purpura, Thrombotic Thrombocytopenic ; Splenomegaly ; Thrombotic Microangiopathies

BACKGROUND/AIMS: Patients with Hansen’s disease are the most vulnerable to hepatitis C. However, no data on the treatment efficacy of direct-acting antiviral agents (DAAs) are available in this group. Therefore, we elucidated the prevalence and clinical outcomes of hepatitis C in persons affected by leprosy in Sorokdo, Jeollanam-do, Korea. METHODS: We retrospectively included 50 leprosy patients with positive hepatitis C virus (HCV) RNA test results (group A) hospitalized at the Sorokdo National Hospital from May 2016 to March 2018 and 73 patients with chronic hepatitis C who were treated with DAAs at the Chonnam National University Hospital (group B) from May 2016 to December 2017. RESULTS: Overall, at the Sorokdo National Hospital, positive HCV antibody and HCV RNA rates were 18.4% and 11.0%, respectively. The mean participant age was 76.5±7 years, and 58% of participants were men. The genotypes were type 1b in 44% (22 out of 50) and type 2 in 56% (28 out of 50). Sustained virologic response was achieved at a rate of 95.5% (21/22) in genotype 1b and 92.9% (26/28) in genotype 2 patients. Ribavirin-induced hemolytic anemia occurred in 57.1% (16/28) of patients with genotype 2. Among these, 28.5% (8/28) received blood transfusions. CONCLUSIONS: Treatment efficacy was not different between the leprosy-affected population and the general population. However, severe ribavirin-induced hemolytic anemia requiring transfusion was present in 28.5% of genotype 2 patients. Therefore, we suggest ribavirin-free DAAs for the treatment of genotype 2 hepatitis C in leprosy-affected persons in the future.
Anemia, Hemolytic ; Antiviral Agents ; Blood Transfusion ; Genotype ; Hepacivirus ; Hepatitis C ; Hepatitis C, Chronic ; Hepatitis, Chronic ; Humans ; Jeollanam-do ; Korea ; Leprosy ; Male ; Prevalence ; Retrospective Studies ; RNA ; Treatment Outcome

Autoimmune hemolytic anemia is a disease characterized with destruction of erythrocytes as a result of antibody produce against patient's own erythrocytes and anemia. Autoimmune hemolytic anemia can be roughly stratified into two groups according to serological features and secondary causes including drugs induced hemolytic anemia. Drugs induced autoimmune hemolytic anemia is very rare in pediatric patients. Even though hematological side effects such as leucopenia, agranulocytosis, eosinophilia, thrombocytopenic purpura and aplastic anemia might occur due to psychotropic drug use; to the best of our knowledge there is no autoimmune hemolytic anemia case due to quetiapine, an atypical antipsychotics, in literature. We hereby describe the first child case of autoimmune hemolytic anemia during quetiapine treatment.We also are pointing out that one should keep in mind serious hematological side effects with atypical antipsychotic drug use with this case report.
Agranulocytosis ; Anemia ; Anemia, Aplastic ; Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune* ; Antipsychotic Agents ; Child* ; Eosinophilia ; Erythrocytes ; Humans ; Purpura, Thrombocytopenic ; Quetiapine Fumarate*

Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) is one of the causes of atypical hemolytic uremic syndrome, and increasingly reported. They are more severe and leave more long-term sequelae than more prevalent, typical hemolytic uremic syndrome. But it is not so easy to diagnose SpHUS for several reasons (below), and there was no diagnostic criteria of consensus. A 18 month-old-girl with sudden onset of oliguria and generalized edema was admitted through the emergency room. She had pneumonia with pleural effusion and laboratory findings of HUS, DIC, and positive direct Coombs' test. As DIC or SpHUS was suspected, we started to treat her with broad spectrum antibiotics, transfusion of washed RBC and replacement of antithrombin III. On the 3rd day, due to severe hyperkalemia and metabolic acidosis, continuous renal replacement therapy (CRRT) was started. She showed gradual improvement in 4 days on CRRT and discharged in 16 days of hospital care. At the follow up to one year, she has maintained normal renal function without proteinuria and hypertension. We report this case with review of articles including recently suggested diagnostic criteria of SpHUS.
Acidosis ; Anti-Bacterial Agents ; Antithrombin III ; Child ; Consensus ; Coombs Test ; Dacarbazine* ; Disseminated Intravascular Coagulation ; Edema ; Emergency Service, Hospital ; Follow-Up Studies ; Hemolytic-Uremic Syndrome* ; Humans ; Hyperkalemia ; Hypertension ; Oliguria ; Pleural Effusion ; Pneumonia ; Proteinuria ; Renal Replacement Therapy ; Streptococcus pneumoniae*

Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) is one of the causes of atypical hemolytic uremic syndrome, and increasingly reported. They are more severe and leave more long-term sequelae than more prevalent, typical hemolytic uremic syndrome. But it is not so easy to diagnose SpHUS for several reasons (below), and there was no diagnostic criteria of consensus. A 18 month-old-girl with sudden onset of oliguria and generalized edema was admitted through the emergency room. She had pneumonia with pleural effusion and laboratory findings of HUS, DIC, and positive direct Coombs' test. As DIC or SpHUS was suspected, we started to treat her with broad spectrum antibiotics, transfusion of washed RBC and replacement of antithrombin III. On the 3rd day, due to severe hyperkalemia and metabolic acidosis, continuous renal replacement therapy (CRRT) was started. She showed gradual improvement in 4 days on CRRT and discharged in 16 days of hospital care. At the follow up to one year, she has maintained normal renal function without proteinuria and hypertension. We report this case with review of articles including recently suggested diagnostic criteria of SpHUS.
Acidosis ; Anti-Bacterial Agents ; Antithrombin III ; Child ; Consensus ; Coombs Test ; Dacarbazine* ; Disseminated Intravascular Coagulation ; Edema ; Emergency Service, Hospital ; Follow-Up Studies ; Hemolytic-Uremic Syndrome* ; Humans ; Hyperkalemia ; Hypertension ; Oliguria ; Pleural Effusion ; Pneumonia ; Proteinuria ; Renal Replacement Therapy ; Streptococcus pneumoniae*

To study the hemolytic effect of polyphyllin II (PP II) mediated by anion channel protein and glucose transporter 1 (GLUT1), in order to initially reveal its hemolytic mechanism in vitro. In the experiment, the spectrophotometric method was adopted to detect the hemolysis of PP II in vitro and the effect of anion channel-related solution and blocker, glucose channel-related inhibitor and multi-target drugs dehydroepiandrosterone (DHEA) and diazepam on the hemolysis of PP II. The scanning electron microscope and transmission electron microscope were used to observe the effect of PP II on erythrocyte (RBC) morphology. The results showed that PP II -processed blood cells were severely deformed into spherocytes, acanthocyturia and vesicae. According to the results of the PP II hemolysis experiment in vitro, the anion hypertonic solution LiCl, NaHCO3, Na2SO4 and PBS significantly inhibited the hemolysis induced by PP II (P < 0.05), while blockers NPPB and DIDS remarkably promoted it (P < 0.01). Hyperosmotic sodium chloride, fructose and glucose at specific concentrations notably antagonized the hemolysis induced by PP II (P < 0.05). The glucose channel inhibitor Cytochalasin B and verapamil remarkably antagonized the hemolysis induced by PP II (P < 0.01). The hemolysis induced by PP II could also be antagonized by 1 gmol x L(1) diazepam and 100 μmol x L(-1) DHEA pretreated for 1 min (P < 0.01). In conclusion, the hemolytic mechanism of PP II in vitro may be related to the increase in intracellular osmotic pressure and rupture of erythrocytes by changing the anion channel transport activity, with GLUT1 as the major competitive interaction site.
Animals ; Diosgenin ; pharmacology ; Drugs, Chinese Herbal ; pharmacology ; Erythrocytes ; cytology ; drug effects ; Hemolysis ; drug effects ; Hemolytic Agents ; pharmacology ; Sheep

Adult ; Anemia, Hemolytic ; diagnosis ; drug therapy ; Anti-Bacterial Agents ; therapeutic use ; Humans ; Male ; Mycoplasma pneumoniae ; pathogenicity ; Pneumonia, Mycoplasma ; diagnosis ; drug therapy

Drug-induced immune haemolytic anaemia (DIIHA) is extremely rare. We herein report a case of life-threatening DIIHA due to levofloxacin. This is the second case reported in the literature. A 51-year-old woman presented with complaints of fatigue after 4-5 days of levofloxacin therapy for a lung infection. At presentation, she was found to have haemolysis with a positive Coombs test and IgG autoantibodies. Levofloxacin was identified as the probable culprit, using the Naranjo adverse drug reaction probability scale. Upon discontinuation of the drug and initiation of steroids, the patient's haematological parameters stabilised. Diagnosis of DIIHA is made through a history of intake of levofloxacin, clinical and laboratory features of haemolysis and a positive Coombs test. An autoantibody screen is most commonly positive for warm antibodies (IgG type). It is essential for clinicians to recognise this rare complication caused by a commonly prescribed medication, discontinue the offending drug and initiate treatment.
Anemia, Hemolytic ; chemically induced ; Anti-Bacterial Agents ; adverse effects ; therapeutic use ; Autoantibodies ; blood ; Female ; Fluoroquinolones ; adverse effects ; Hemolysis ; Humans ; Immunoglobulin G ; blood ; Levofloxacin ; adverse effects ; Male ; Middle Aged ; Steroids ; therapeutic use

While imported falciparum malaria has been increasingly reported in recent years in Korea, clinicians have difficulties in making a clinical diagnosis as well as in having accessibility to effective anti-malarial agents. Here we describe an unusual case of imported falciparum malaria with severe hemolytic anemia lasting over 2 weeks, clinically mimicking a coinfection with babesiosis. A 48-year old Korean man was diagnosed with severe falciparum malaria in France after traveling to the Republic of Benin, West Africa. He received a 1-day course of intravenous artesunate and a 7-day course of Malarone (atovaquone/proguanil) with supportive hemodialysis. Coming back to Korea 5 days after discharge, he was readmitted due to recurrent fever, and further treated with Malarone for 3 days. Both the peripheral blood smears and PCR test were positive for Plasmodium falciparum. However, he had prolonged severe hemolytic anemia (Hb 5.6 g/dl). Therefore, 10 days after the hospitalization, Babesia was considered to be potentially coinfected. A 7-day course of Malarone and azithromycin was empirically started. He became afebrile within 3 days of this babesiosis treatment, and hemolytic anemia profiles began to improve at the completion of the treatment. He has remained stable since his discharge. Unexpectedly, the PCR assays failed to detect DNA of Babesia spp. from blood. In addition, during the retrospective review of the case, the artesunate-induced delayed hemolytic anemia was considered as an alternative cause of the unexplained hemolytic anemia.
Anemia, Hemolytic/chemically induced/*etiology/*pathology ; Anti-Bacterial Agents/therapeutic use ; Antimalarials/therapeutic use ; Artemisinins/adverse effects/therapeutic use ; Atovaquone/therapeutic use ; Azithromycin/therapeutic use ; Babesiosis/complications/*diagnosis/drug therapy/*pathology ; Benin ; Blood/parasitology ; Coinfection/diagnosis/pathology ; Drug Combinations ; France ; Humans ; Korea ; Malaria, Falciparum/complications/*diagnosis/drug therapy/*pathology ; Male ; Middle Aged ; Plasmodium falciparum/*isolation & purification ; Proguanil/therapeutic use ; Travel ; Treatment Outcome

Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report herein a case of AHA complicated by both PRCA and AIHA. A 49-year-old female with a diagnosis of AHA presented with severe anemia (hemoglobin level, 6.9 g/dL) during her clinical course. A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. Her anemia and liver function test were completely recovered by 9 months after the initial presentation. We review the clinical features and therapeutic strategies for this rare case of extrahepatic manifestation of AHA.
Acute Disease ; Adult ; Anemia, Hemolytic, Autoimmune/*complications/*diagnosis/drug therapy ; Antineoplastic Agents, Hormonal/therapeutic use ; Bone Marrow/pathology ; Female ; Hepatitis A/*complications/*diagnosis ; Humans ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Red-Cell Aplasia, Pure/*complications/*diagnosis/drug therapy ; Treatment Outcome ; Young Adult