-
Blood Advances Feb 2024
Topics: Humans; Anemia, Hemolytic, Autoimmune
PubMed: 37883797
DOI: 10.1182/bloodadvances.2023011264 -
The American Journal of Case Reports Feb 2022BACKGROUND Autoimmune hemolytic anemia and immune thrombocytopenia are rare complications of brucellosis; only a few cases have been reported in the literature. While... (Review)
Review
BACKGROUND Autoimmune hemolytic anemia and immune thrombocytopenia are rare complications of brucellosis; only a few cases have been reported in the literature. While pancytopenia is common and was reported in Saudi Arabia, the description of autoimmune hemolytic anemia or immune thrombocytopenia has not yet been reported in the kingdom. Hematological complication usually requires supportive treatment, and it is expected to improve with the initiation of antimicrobial therapy for brucellosis. There are few reports on the treatment of patients that fail to improve with conventional therapy. CASE REPORT A 46-year-old previously healthy Saudi woman was admitted to our hospital after multiple visits to the emergency department with chief concerns of fever and fatigability for 30 days. The examination was remarkable only for fever of 38.4°C and tender hepatomegaly. Laboratory tests upon admission were significant of pancytopenia, with a white blood count of 3×10⁹/L, hemoglobin of 8.1 g/dL, platelet of 13×10⁹/L, moderate hyponatremia, hypokalemia, and metabolic acidosis. Tuberculosis was ruled out and pan-sensitive brucellosis was diagnosed. She was started on standard antimicrobial therapy without significant improvement. Further testing revealed Coomb's-positive hemolytic anemia and possible immune-mediated severe thrombocytopenia. She was treated with glucocorticoids and intravenous immunoglobulin, with dramatic response. CONCLUSIONS Autoimmune-mediated destruction of blood lines in brucellosis is rare. It should be sought as a potential diagnosis in case of persistent anemia and/or thrombocytopenia that is severe or fails to improve with proper antimicrobial coverage. Early involvement of hematologists and initiation of glucocorticoid with or without intravenous immunoglobulin is crucial.
Topics: Anemia, Hemolytic; Anemia, Hemolytic, Autoimmune; Brucellosis; Female; Humans; Middle Aged; Saudi Arabia; Thrombocytopenia
PubMed: 35210389
DOI: 10.12659/AJCR.935187 -
BMC Urology Jul 2015Autoimmune hemolytic anemia (AIHA) is hemolytic anemia characterized by autoantibodies directed against red blood cells. AIHA can be induced by hematological neoplasms... (Review)
Review
BACKGROUND
Autoimmune hemolytic anemia (AIHA) is hemolytic anemia characterized by autoantibodies directed against red blood cells. AIHA can be induced by hematological neoplasms such as malignant lymphoma, but is rarely observed in the urological field. We report a case of renal urothelial cancer inducing Coombs-positive warm AIHA and severe thrombocytopenia that was responsive to nephroureterectomy.
CASE PRESENTATION
A 52-year-old man presented with a 1-month history of general weakness and dizziness. Hemoglobin level was 4.2 g/dL, and direct and indirect Coombs tests both yielded positive results. Abdominal computed tomography revealed huge left hydronephrosis due to a renal pelvic tumor measuring 4.0 x 4.0 x 3.0 cm, and renal regional lymph-node involvement was also observed and suspected as metastasis. Corticosteroid therapy was administered, and nephroureterectomy was performed. After surgical resection, the hemoglobin level gradually normalized, and direct and indirect Coombs tests yielded negative results. We thus diagnosed warm AIHA associated with renal urothelial cancer.
CONCLUSION
To the best of our knowledge, this represents the first report of AIHA associated with renal urothelial cancer and severe thrombocytopenia responsive to nephroureterectomy. Renal urothelial cancer needs to be included in the differential diagnoses for warm AIHA, and nephroureterectomy represents a treatment option for AIHA.
Topics: Anemia, Hemolytic, Autoimmune; Carcinoma, Transitional Cell; Diagnosis, Differential; Humans; Kidney Neoplasms; Male; Middle Aged; Nephrectomy; Treatment Outcome
PubMed: 26215157
DOI: 10.1186/s12894-015-0071-0 -
Saudi Medical Journal Apr 2019Ovarian teratoma is a rare cause of autoimmune hemolytic anemia (AIHA) by warm antibodies, resistant to corticosteroid therapy. This also implies that ovarian teratoma...
Ovarian teratoma is a rare cause of autoimmune hemolytic anemia (AIHA) by warm antibodies, resistant to corticosteroid therapy. This also implies that ovarian teratoma should be included in the differential diagnosis of AIHA, whether or not associated with pregnancy. We present a case of a primigravida who presented with ovarian dermoid cysts and AIHA at 24 weeks of gestation. The patient received corticosteroids, intravenous immunoglobulin, rituximab, and multiple blood transfusions, with no significant improvement. Hemoglobin levels returned to normal only after laparoscopic ovarian cystectomy. Autoimmune hemolytic anemia caused by dermoid cyst is a rare condition especially in pregnancy. However, in light of similar case reports and review of the existing literature, we conclude that surgical excision should be considered when AIHA and ovarian teratoma coexist.
Topics: Adult; Anemia, Hemolytic, Autoimmune; Female; Humans; Ovarian Neoplasms; Ovariectomy; Ovary; Postpartum Period; Pregnancy; Pregnancy Complications, Hematologic; Pregnancy Complications, Neoplastic; Pregnancy Outcome; Teratoma; Treatment Outcome; Young Adult
PubMed: 30957135
DOI: 10.15537/smj.2019.4.24107 -
Frontiers in Immunology 2022Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency (PID). CVID is a heterogenous condition and clinical manifestations may... (Review)
Review
Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency (PID). CVID is a heterogenous condition and clinical manifestations may vary from increased susceptibility to infections to autoimmune manifestations, granulomatous disease, polyclonal lymphoproliferation, and increased risk of malignancy. Autoimmune manifestations may, at times, be the first and only clinical presentation of CVID, resulting in diagnostic dilemma for the treating physician. Autoimmune cytopenias (autoimmune haemolytic anaemia and/or thrombocytopenia) are the most common autoimmune complications seen in patients with CVID. Laboratory investigations such as antinuclear antibodies, direct Coomb's test and anti-platelet antibodies may not be useful in patients with CVID because of lack of specific antibody response. Moreover, presence of autoimmune cytopenias may pose a significant therapeutic challenge as use of immunosuppressive agents can be contentious in these circumstances. It has been suggested that serum immunoglobulins must be checked in all patients presenting with autoimmune cytopenia such as immune thrombocytopenia or autoimmune haemolytic anaemia. It has been observed that patients with CVID and autoimmune cytopenias have a different clinical and immunological profile as compared to patients with CVID who do not have an autoimmune footprint. Monogenic defects have been identified in 10-50% of all patients with CVID depending upon the population studied. Monogenic defects are more likely to be identified in patients with CVID with autoimmune complications. Common genetic defects that may lead to CVID with an autoimmune phenotype include and In this review, we update on recent advances in pathophysiology and management of CVID with autoimmune cytopenias.
Topics: Adaptor Proteins, Signal Transducing; Anemia, Hemolytic, Autoimmune; Antibodies, Antinuclear; Common Variable Immunodeficiency; Humans; Phosphatidylinositol 3-Kinases; Thrombocytopenia
PubMed: 35795667
DOI: 10.3389/fimmu.2022.869466 -
Clinical and Molecular Hepatology Jun 2014Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report... (Review)
Review
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.
Topics: Acute Disease; Adult; Anemia, Hemolytic, Autoimmune; Antineoplastic Agents, Hormonal; Bone Marrow; Female; Hepatitis A; Humans; Male; Middle Aged; Prednisolone; Red-Cell Aplasia, Pure; Treatment Outcome; Young Adult
PubMed: 25032187
DOI: 10.3350/cmh.2014.20.2.204 -
Clinical & Developmental Immunology 2013Warm autoimmune hemolytic anemia (WAIHA) is one of four clinical types of autoimmune hemolytic anemia (AIHA), with the characteristics of autoantibodies maximally active... (Review)
Review
Warm autoimmune hemolytic anemia (WAIHA) is one of four clinical types of autoimmune hemolytic anemia (AIHA), with the characteristics of autoantibodies maximally active at body temperature. It produces a variable anemia-sometimes mild and sometimes severe. With respect to the absence or presence of an underlying condition, WAIHA is either idiopathic (primary) or secondary, which determines the treatment strategies in practice. Conventional treatments include immune suppression with corticosteroids and, in some cases, splenectomy. In recent years, the number of clinical studies with monoclonal antibodies and immunosuppressants in the treatment of WAIHA increased as the knowledge of autoimmunity mechanisms extended. This thread of developing new tools of treating WAIHA is well exemplified with the success in using anti-CD20 monoclonal antibody, Rituximab. Following this success, other treatment methods based on the immune mechanisms of WAIHA have emerged. We reviewed these newly developed immunotherapy treatments here in order to provide the clinicians with more options in selecting the best therapy for patients with WAIHA, hoping to stimulate researchers to find more novel immunotherapy strategies.
Topics: Anemia, Hemolytic, Autoimmune; Animals; Antibodies, Monoclonal; Humans; Immunosuppressive Agents; Immunotherapy
PubMed: 24106518
DOI: 10.1155/2013/561852 -
Blood Aug 2013Cold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia. We reviewed the clinical and pathologic... (Review)
Review
Cold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia. We reviewed the clinical and pathologic features, prognosis, and management in the literature and describe our institutional experience to improve strategies for accurate diagnosis and treatment. Retrospective analysis identified 89 patients from our institution with cold agglutinin disease from 1970 through 2012. Median age at symptom onset was 65 years (range, 41 to 83 years), whereas the median age at diagnosis was 72 years (range, 43 to 91 years). Median survival of all patients was 10.6 years, and 68 patients (76%) were alive 5 years after the diagnosis. The most common symptom was acrocyanosis (n = 39 [44%]), and many had symptoms triggered by cold (n = 35 [39%]) or other factors (n = 20 [22%]). An underlying hematologic disorder was detected in 69 patients (78%). Thirty-six patients (40%) received transfusions during their disease course, and 82% received drug therapy. Rituximab was associated with the longest response duration (median, 24 months) and the lowest proportion of patients needing further treatment (55%). Our institution's experience and review of the literature confirms that early diagnostic evaluation and treatment improves outcomes in cold agglutinin disease.
Topics: Anemia, Hemolytic, Autoimmune; Disease Management; Humans; Prognosis; Retrospective Studies
PubMed: 23757733
DOI: 10.1182/blood-2013-02-474437 -
Archivum Immunologiae Et Therapiae... Oct 2013Autoimmune hemolytic anemia (AIHA) is an infrequent group of diseases defined by autoantibody mediated red blood cell destruction. Correct diagnosis and classification... (Review)
Review
Autoimmune hemolytic anemia (AIHA) is an infrequent group of diseases defined by autoantibody mediated red blood cell destruction. Correct diagnosis and classification of this condition are essential to provide appropriate treatment. AIHA is divided into warm and cold types according to the characteristics of the autoantibody involved and by the presence of an underlying or associated disorder into primary and secondary AIHA. Due to its low frequency, treatment for AIHA is largely based on small prospective trials, case series, and empirical observations. This review describes in detail the different treatment approaches for autoimmune hemolytic anemia. Warm antibody type AIHA should be treated with steroids, to which most patients respond, although relapse can occur and maintenance doses are frequently required. Splenectomy is an effective second line treatment and can provide long-term remission without medication. Rituximab is a useful alternative for steroid refractory patients, those requiring high maintenance doses and unfavorable candidates for surgery. Promising therapeutic modifications with this monoclonal antibody are emerging including drug combinations, lower doses, and long-term use. Primary cold agglutinin disease has been recognized as having a lymphoproliferative monoclonal origin. It is unresponsive to both steroids and splenectomy. Rituximab is currently the best therapeutic alternative for this condition, and several treatment regimens are available with variable responses.
Topics: Adrenal Cortex Hormones; Anemia, Hemolytic, Autoimmune; Animals; Antibodies, Monoclonal, Murine-Derived; Humans; Immunosuppression Therapy; Recurrence; Rituximab; Splenectomy
PubMed: 23689532
DOI: 10.1007/s00005-013-0232-3 -
Hematology/oncology and Stem Cell... Dec 2022Lymphopenia, thrombocytopenia, and elevated D-dimer and ferritin levels are frequently reported in patients with severe coronavirus disease 2019 (COVID-19). Here we...
BACKGROUND
Lymphopenia, thrombocytopenia, and elevated D-dimer and ferritin levels are frequently reported in patients with severe coronavirus disease 2019 (COVID-19). Here we report a case of cold agglutinin disease (CAD), autoimmune hemolytic anemia (AIHA), and pulmonary embolism as a presentation of COVID-19 infection.
CASE REPORT
A 51-year-old African-American woman presented to the emergency room with fever and shortness of breath. She was tachycardic, febrile, and had an oxygen saturation of 88% on room air. Laboratory studies showed hemoglobin (Hb) 5.1 g/dL, D-dimer 4.55 μg/mL, and C-reactive protein 12.3 mg/dL. Computed tomography scan of the chest showed acute pulmonary embolism involving the bilateral lower lobe segmental branches. Her influenza test was negative, but her SARS-CoV-2 test returned positive. Due to severe anemia, she was not started on any anticoagulation. Haptoglobin was low. Direct antiglobulin test returned positive for anticomplement and negative for anti-immunoglobulin G. Cold agglutinin titer was 80. Mycoplasma, Epstein-Barr virus, parvovirus, human immunodeficiency viruses, and acute hepatitis screen were negative. Abdominal and pelvic computed tomography showed a normal liver and spleen without lymphadenopathy. Peripheral blood smear showed red blood cell agglutination. On Day 2, she became hypoxic requiring 6 L oxygen. Since her Hb remained stable, she was started on low-intensity unfractionated heparin. Inflammatory markers subsequently improved and she was weaned off oxygen. Her Hb remained stable at 9 g/dL and she was discharged home. After 2 weeks, her Hb increased to 11 g/dL.
CONCLUSION
As exemplified in this case report, COVID-19 infection can lead to thromboembolism, CAD, and AIHA and it should be recognized as a potential etiology to such rare diseases.
Topics: Female; Humans; Middle Aged; COVID-19; Anemia, Hemolytic, Autoimmune; Heparin; Epstein-Barr Virus Infections; SARS-CoV-2; Herpesvirus 4, Human; Pulmonary Embolism; Thrombocytopenia; Fever
PubMed: 32645300
DOI: 10.1016/j.hemonc.2020.06.005