Autoimmune Hemolytic Anemia

Pirofsky, Bernard

doi:10.1016/b978-0-12-596920-8.50025-1

Cited by 22 publications

(33 citation statements)

References 12 publications

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“…Various mechanisms of AIHA have been postulated, including absorption of immune complexes and complement cross-reacting antigen and a true autoimmune state with possible loss of tolerance secondary to the infectious organism. 8 Our case, similar to the case reported by Siribaddana and Wijesundera, 6 who described a case of tuberculous lymphadenitis with positive direct and indirect antiglobulin tests, was due to cold agglutinins. The two earlier cases, 4,5 however, only demonstrated evidence of hemolysis with a positive antiglobulin test in patients with genitourinary and pulmonary tuberculosis, respectively, who responded to anti-tuberculous therapy.…”

Section: Discussionsupporting

confidence: 71%

Autoimmune Hemolytic Anemia Associated with Intestinal Tuberculosis

2002

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Tuberculosis, a common disease in the developing world and a resurging problem in the developed world, is associated with numerous hematological manifestations. The most common manifestation is normochromic normocytic anemia of chronic disease. Hemolytic anemia is rare, and there are only a few previously reported cases. We report a case of autoimmune hemolytic anemia (AIHA) associated with intestinal tuberculosis which responded to antituberculous therapy alone. To our knowledge, this is the fourth case to be reported in the literature. Case ReportA 21-year-old Indonesian housemaid presented to the Emergency Department with a one-week history of vomiting and vague abdominal pain. She had a 4-week history of diarrhea and weight loss of about 3 kg associated with intermittent fever but no night sweats. Two weeks prior to presentation, she had had an upper respiratory tract infection, which resolved spontaneously. She had not been receiving any medications on presentation, and a review of her systems had been unremarkable.On examination, the patient was found to be pale, febrile (temp. 38.5°c) and mildly jaundiced. Examination of the abdomen revealed mild tenderness in the right iliac fossa but no palpable masses. The remainder of her examination was within normal limits.Laboratory results were significant for the following (Table 1): WBC 9.8x109/L (59% neutrophils, 31% lymphocytes, 7% monocytes, 3% eosinophils); Hb 5.1 g/dL; platelets 314x10 3 ; MCV 110 f1; MCH 36.2 pg; reticulocyte count 15.4%, and erythrocyte sedimentation rate (ESR) 150 mm/hr. Antiglobulin tests (both direct and indirect) were strongly positive. Cold agglutinins were detected as the responsible antibodies. Unfortunately, the thermal amplitudes of the cold agglutinin could not be determined. Liver function tests (LFT) revealed total bilirubin of 50 μmol/L (normal range up to 17 μmol/L), indirect bilirubin 40 μmol/L, and lactate dehydrogenase levels of 1480 m/L (normal range 230-460 m/L). The rest of the LFT, urea and electrolytes were normal. Peripheral blood smear showed moderate polychromasia and macrocytosis.Serology for mycoplasma, respiratory viruses, human immunodeficiency virus and cytomegalovirus were negative, as was the result of Paul-Bunnell test. Antinuclear antibody and anti-DNA antibody tests were also negative. Hemoglobin electrophoresis was normal (A 97.5% AI, 2.5% A2), and serum iron, TIBC, folate and B 12 levels were within normal limits. Bone marrow examination revealed normal bone marrow with normoblastic hyperplasia.Blood, urine and stool cultures were all negative. Mantoux test with 5 IU of PPD was strongly positive (30 mm induration). Upper gastrointestinal tract endoscopy was normal. Ultrasound scan of the abdomen revealed hypoechoic nodules adjacent to the lower one-third of the inferior vena cava (IVC), suggesting enlarged lymph nodes. CT scan of the abdomen confirmed the presence of enlarged lymph nodes (2x3 cm) adjacent to the lower one-third of the IVC, in addition to thickening of the walls of the cecum and termina...

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Section: Discussionsupporting

confidence: 71%

Autoimmune Hemolytic Anemia Associated with Intestinal Tuberculosis

2002

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“…Although patients with malignant lymphoma often show a positive Coombs' test, they rarely develop AIHA (5). In addition, a previous study showed that only about 4% of AIHA patients had myeloma (5), while other reports have stated that myeloma is rarely associated with hemolytic anemia (6, 7).…”

Section: Discussionmentioning

confidence: 99%

“…Patients with secondary AIHA often have underlying collagen diseases or hematologic malignancies (5 and it has been reported to precede the onset of chronic lymphocytic leukemia. Although patients with malignant lymphoma often show a positive Coombs' test, they rarely develop AIHA (5).…”

Section: Discussionmentioning

confidence: 99%

Multiple Myeloma Complicated by Autoimmune Hemolytic Anemia

et al. 2004

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“…However, progress in treatment has been much slower. [3][4][5][6][7][8] Therapy has been reviewed by several investigators, [8][9][10][11][12][13][14][15] but no treatment guidelines have yet been published. …”

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How I treat autoimmune hemolytic anemias in adults

Lechner

Jäger

2010

Blood

277

246

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Autoimmune hemolytic anemia is a heterogeneous disease with respect to the type of the antibody involved and the absence or presence of an underlying condition. Treatment decisions should be based on careful diagnostic evaluation. Primary warm antibody autoimmune hemolytic anemias respond well to steroids, but most patients remain steroid-dependent, and many require second-line treatment. Currently, splenectomy can be regarded as the most effective and best-evaluated second-line therapy, but there are still only limited data on longterm efficacy and adverse effects. The monoclonal anti-CD20 antibody rituximab is another second-line therapy with documented short-term efficacy, but there is limited information on long-term efficacy and side effects. The efficacy of immunosuppressants is poorly evaluated. Primary cold antibody autoimmune hemolytic anemias respond well to rituximab but are resistant to steroids and splenectomy. The most common causes of secondary autoimmune hemolytic anemias are malignancies, immune diseases, or drugs. They may be treated in a way similar to primary autoimmune hemolytic anemias, by immunosuppressants or by treatment of the underlying disease. IntroductionAutoimmune hemolytic anemia (AIHA) is a rare disease. In a recent population-based study 1 the incidence was 0.8/100 000/year, but the prevalence is 17/100 000. 2 Primary (idiopathic) AIHA is less frequent than secondary AIHA. Secondary cases are often challenging because not only AIHA but also the underlying disease(s) must be diagnosed and treated. AIHA is essentially diagnosed in the laboratory, and considerable improvement has been made in this field. However, progress in treatment has been much slower. [3][4][5][6][7][8] Therapy has been reviewed by several investigators, [8][9][10][11][12][13][14][15] but no treatment guidelines have yet been published. DiagnosisThe diagnosis of AIHA is usually straightforward and made on the basis of the following laboratory findings: normocytic or macrocytic anemia, reticulocytosis, low serum haptoglobin levels, elevated lactate dehydrogenase (LDH) level, increased indirect bilirubin level, and a positive direct antiglobulin test with a broad-spectrum antibody against immunoglobulin and complement ( Figure 1). However, there are pitfalls, particularly in secondary cases, because not always are all of the typical laboratory findings of AIHA present. 15 Two pieces of information are of utmost importance for the clinician to make an appropriate treatment decision: (1) What type of the antibody is involved? (2) Is the AIHA primary or secondary? The type of antibody can be identified with the use of monospecific antibodies to immunoglobulin G (IgG) and C3d. When the red cells are coated with IgG or IgG plus C3d, the antibody is usually a warm antibody (warm antibody AIHA [WAIHA]). When the red cells are coated with C3d only, the antibody is often but not always a cold antibody. For definite diagnosis of a cold antibody AIHA (CAIHA), the cold agglutinin titer should be markedly elevated (Ͼ 1:512)...

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Autoimmune Hemolytic Anemia

Cited by 22 publications

References 12 publications

Autoimmune Hemolytic Anemia Associated with Intestinal Tuberculosis

Autoimmune Hemolytic Anemia Associated with Intestinal Tuberculosis

Multiple Myeloma Complicated by Autoimmune Hemolytic Anemia

How I treat autoimmune hemolytic anemias in adults

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