Journal of Blood Disorders & Transfusion

Cold agglutinin autoimmune hemolytic anemia: A surprising presentation of high-grade diffuse B cell lymphoma

2^nd International Conference on Hematology & Blood Disorders

September 29-October 01, 2014 DoubleTree by Hilton Baltimore-BWI Airport, USA

Deobrat Mallick, Jecmenica Mladen, Hasan Zerti and Evan Ruppell

Posters: J Blood Disorders Transf

Abstract:

Introduction: Cold agglutinin disease is a rare, most often affecting females in the seventh decade of life, with an incidence of one case per million people per year. It is characterized by the presence of IgM antibodies (rarely IgA or IgG) directed against polysaccharide antigens on the red blood cell surface. These antibodies transiently bind erythrocytes in the colder extremities, generally below 30 degrees C. They dissociate after returning centrally, but frequently sublytic quantities of the opsonin C3b will be bound the erythrocyte surface. This interaction causes the affected blood cells to be removed by the spleen, liver, and bone marrow. Hemolysis is not necessarily severe. Cold agglutinin disease is often caused by infection by pathogens such as Mycoplasma pneumoniae , cytomegalovirus, influenza virus, or Ebstein-Barr Virus.It can also reflect the presence of an underlying lymphoproliferative disorder, encompassing a wide range of disorders. Case: The patient was a 91 year old female presented to hospital after her outpatient workup showed sever anemia and a positive Stool for occult blood test. She was recently started on Naproxen and steroid for back pain by her PCP. Her hemoglobin upon presentation to the emergency department was 6.1 g/dl, for which she received one unit of PRBCs.She underwent EGD and a bleedinggastric lesion was identified. Biopsy was taken from the lesion. The cause of anemia at this time was suspected to be GI bleed; however a workup of the patient's anemia also raised the possibility of hemolytic anemia with evidence high indirect bilirubin and LDH. Haptoglobin was low normal. Further workup for auto-immune hemolysis with direct Coombs test was positive for C3B and 3D, but Negative for IgG. Hematology evaluation with cold agglutinin titers and thermal amplitude test revealed a cold agglutinin autoimmune hemolytic anemia secondary to Anti-I antibody. Meanwhile, thegastric biopsy was analyzed and was found to contain high-grade diffuse B cell lymphoma. CT showed a large retroperitoneal soft tissue mass, most likely lymphadenopathy, displacing nearby organs and extending into the left psoas and L3 vertebral body. An MRI found extension of this mass into the neural foramina on the left at L2-3 & L3-4 and on the right at L3-4. There were also linear signal alterations consistent with pathologic fracture in the body of L3.After discussion with the patient and her family,chemoimmunotherapywas started. Rituximab was begun after hepatitis B serology was found to be negative. Oncovin, adriamycin, and dose-reduced cylcophosphamide were added to her regimen 2 days later. Discussion: Treatment of mildly symptomatic cold agglutinin disease (e.g., mild degrees of acrocyanosis or anemia) is mainly supportive, and consists of avoidance of exposure to cold and additional sources of complement. In the presence of severe signs and symptoms, treatment with rituximab with or without fludarabinewas recommended. When there is evidence for its presence, treatment of an underlying lymphoma is often successful in reducing signs and symptoms of cold agglutinin disease. Conclusion: The many pathogeneses of anemia can be overwhelming and may prompt the physician to settle for the first explanation that comes to hand. However the astute clinicianfaced with cold agglutinin autoimmune hemolytic anemia should always consider underlying lymphoproliferative disorder in addition to infectious causes.

Biography :

Deobrat Mallickis a second year Internal medicine resident at Wright Center for Graduate medical education (WCGME) at Scranton. He has done his Medical school from Guwahati Medical College, Assam, India in 1997. He hasbegunhis Post-graduate training program (MD) in Internal Medicine in the year 1999 at Jiwaji University at Gwalior. In 2004 he had moved to United Kingdom and trained and worked in Emergency Medicine for several years before moving to USA in 2012 to start his residency in Medicine. He has now worked for several years in UK, India and USA. During which period he has worked in both large city hospitals and smaller district general hospitals with different levels of capabilities and this has helped him to hone his clinical knowledge and various skills.