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Rationale: Dengue fever is capable of inciting the formation of transient polyclonal antibodies directed at red blood cell antigens, resulting in complement-mediated hemolysis, leading to intravascular hemolysis and hemoglobinuria. Patient’s concern: A 12-year-old male patient who recovered from dengue fever a week ago had red blood cell agglutination, spherocytes, and engulfment of red blood cells (erythrophagocytosis) by monocytes and neutrophils on routine hematological peripheral blood smear. The unexpected blood smear results prompted the lab physicians to investigate autoimmune hemolytic anemia, which revealed a monospecific positive direct antiglobulin test for complement (C3d, C3b) and the presence of Donath-Landsteiner antibody. Diagnosis: Paroxysmal cold hemoglobinuria (PCH), secondary to dengue fever. Interventions: Oxygen supplements, antibiotics, intravenous immunoglobulins, steroid therapy, and packed cell transfusions were administered. Outcomes: The patient’s condition was improved following the therapy. Lessons: Post-dengue PCH is a rare complication that requires a thorough peripheral smear examination for erythrophagocytosis, as advanced hematology analyzers fail to detect such findings.

The Donath-Landsteiner (DL) hemolytic anemia or paroxysmal cold hemoglobinuria (PCH) is a rare subtype of autoimmune hemolytic anemia (AIHA). We report here a case of a 22-year-old young female who presented with acute onset shortness of breath. Routine hematological investigation revealed erythrophagocytosis and platelet phagocytosis by monocytes in peripheral blood smear, which are rarely reported in AIHA, especially the DL. The unusual blood smear findings directed the pathologists to initiate workup for hemolytic anemia that yielded positive results in direct Coombs test C3d (Complement) and direct DL test. The findings confirmed the case as DL hemolytic anemia. To the best of our knowledge, this is the first report of both erythrophagocytosis and platelet phagocytosis in peripheral blood smear in PCH.

The Donath-Landsteiner (DL) hemolytic anemia or paroxysmal cold hemoglobinuria (PCH) is a rare subtype of autoimmune hemolytic anemia (AIHA). We report here a case of a 22-year-old young female who presented with acute onset shortness of breath. Routine hematological investigation revealed erythrophagocytosis and platelet phagocytosis by monocytes in peripheral blood smear, which are rarely reported in AIHA, especially the DL. The unusual blood smear findings directed the pathologists to initiate workup for hemolytic anemia that yielded positive results in direct Coombs test C3d (Complement) and direct DL test. The findings confirmed the case as DL hemolytic anemia. To the best of our knowledge, this is the first report of both erythrophagocytosis and platelet phagocytosis in peripheral blood smear in PCH. [ABSTRACT FROM AUTHOR]

61-year old male patient was admitted to the hospital with clinical picture of hemolytic anemia with hemoglobinuria. Patient was suspected to have Infectious Mononucleosis (IM) with Auto Immune Hemolytic Anemia (AIHA). DAT was positive with anti-C3d specificity. Donath Landsteiner (DL) test was positive; confirming Paroxysmal Cold Hemoglobinuria (PCH). The final diagnosis was IM with PCH. Patient was managed conservatively and discharged after seven days. DL test specifically detects a biphasic autoantibody (IgG type) that binds to RBCs at cold temperatures, and fixes complement on the RBC membrane. However RBCs are only lysed upon warming to 37C when complement cascade proceeds to completion.

Paroxysmal cold hemoglobinuria is a rare form of autoimmune hemolytic anemia caused by the Donath-Landsteiner autoantibody. The condition is characterized by the presence of an IgG biphasic hemolysin with specificity to the P blood group antigen. The antibody biphasic action may be demonstrated in the Donath-Landsteiner test. While paroxysmal cold hemoglobinuria can be manifested at any age, it typically appears in children following a viral upper respiratory syndrome or immunization, though rarely. This report describes a 23-months old girl presented with 5 days history of fever, erythrocytopenia, leukocytosis and occurrence of dark urine. On admission, the physical examination showed pallor, no scleral icterus, a mild hyperemic throat and no hepatosplenomegaly. The investigations revealed severe anemia with hemoglobin of 44 g/L, increased reticulocyte count (10.67%), elevated lactate dehydrogenase (2603 IU/L), decreased serum haptoglobin (0.159 g/L), normal G6PD. Direct antiglobulin test was positive with C3d and C3c complement components only. Direct and indirect Donath-Landsteiner tests were positive. The girl was treated with a intravenous immunoglobulin infusion and Cefotaxime. She received transfusion of red blood cells, crossmatched, although P antigen untyped. Despite this in vitro serological incompatibility she had a hemoglobin increase. The patient was discharged in stable condition on the seventh day following admission. Paroxysmal cold hemoglobinuria is a hemolytic anemia for which a specific diagnostic test is available. Timely recognition of the disease by pediatricians is crucial as well as the highly skilled hospital blood bank staff performing Donath-Landsteiner testing. [ABSTRACT FROM AUTHOR]

61-year old male patient was admitted to the hospital with clinical picture of hemolytic anemia with hemoglobinuria. Patient was suspected to have Infectious Mononucleosis (IM) with Auto Immune Hemolytic Anemia (AIHA). DAT was positive with anti-C3d specificity. Donath Landsteiner (DL) test was positive; confirming Paroxysmal Cold Hemoglobinuria (PCH). The final diagnosis was IM with PCH. Patient was managed conservatively and discharged after seven days. DL test specifically detects a biphasic autoantibody (IgG type) that binds to RBCs at cold temperatures, and fixes complement on the RBC membrane. However RBCs are only lysed upon warming to 37C when complement cascade proceeds to completion. [ABSTRACT FROM AUTHOR]

anemias and sporadically in few other conditions. Here, we report a case of florid erythrophagocytosis with severe anemia following a viral infection in an 18-year-old girl. Her complete blood count (CBC) revealed hemoglobin of 3.6 gm/dl and a hematocrit of 10%. The peripheral smear showed erythrophagocytosis by neutrophils and rosetting of erythrocytes around neutrophils. The direct Coombs test and direct Donath- Landsteiner tests were positive.

Erythrophagocytosis is a relatively rare observation on blood smears. It has been reported in auto immune hemolytic anemias and sporadically in few other conditions. Here, we report a case of florid erythrophagocytosis with severe anemia following a viral infection in an 18-year-old girl. Her complete blood count (CBC) revealed hemoglobin of 3.6 gm/dl and a hematocrit of 10%. The peripheral smear showed erythrophagocytosis by neutrophils and rosetting of erythrocytes around neutrophils. The direct Coombs test and direct Donath-Landsteiner tests were positive. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the online article "Management of cold haemolytic syndrome," by M.A. Gertz in the June 20, 2008 issue.