Expression of CD47, CD35, CD55 and CD59 on Red Cells from Patients with Warm Autoimmune Hemolytic Anemia.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3738-3738
Author(s):  
Melca O. Barros ◽  
Mihoko Yamamoto ◽  
Maria S. Figueiredo ◽  
Elisa Y. Kimura ◽  
Jose Orlando Bordin
Keyword(s):  
Hemolytic Anemia ◽  
Lupus Erythematosus ◽  
Autoimmune Hemolytic Anemia ◽  
Experimental Studies ◽  
Red Cells ◽  
High Dose ◽  
Healthy Controls ◽  
Life Threatening ◽  
Positive Direct ◽  
Cd59 Expression

Abstract Autoimmune hemolytic anemia (AIHA) is defined as an increased destruction of red cells (RBC) in the presence of anti-RBC autoantibodies. CD47 is an integrin-associated protein expressed on all cells including RBCs. Animal models show that CD47 deficiency contributes to accelerated development of AIHA, while CD35 (CR1- complement receptor 1), CD55 (decay accelerating factor), and CD59 (membrane inhibitor of reactive lysis) are complement inhibitory proteins. Using flow cytometry analysis, in this study we evaluated the expression of CD47, CD35, CD55, and CD59 on RBCs of patients with warm AIHA before any treatment had been initiated. The study population consisted of 12 patients with active AIHA [M:F = 6:6, median age: 32 yrs (3 – 73)], and 20 healthy controls [M:F = 9:11, median age: 36 yrs (25 – 71)]. Ten patients presented idiopathic AIHA while 2 subjects had secondary AIHA (systemic lupus erythematosus and non-Hodgkin’s lymphoma). At presentation the median Hb level was 6.6 mg/dL (range: 2.9 to 10 mg/dL), and the median absolute reticulocyte count was 324 × 109/L (range: 215 to 756 × 109/L). At the time of the analyses, all 12 patients had a positive direct antiglobulin test (DAT), 12 (100%) had IgG on their RBCs, 5 (41.7%) had IgG plus C3, and none had C3 alone. The strength of agglutination of all positive DATs showed a strong reaction. The RBC eluates prepared by a dichloromethane technique from the cell samples were positive in all 12 patients, but the retrieved autoantibodies were pan-reactive showing no specific reactivity. The mean fluorescence intensity (MFI) of the expression of CD47, CD35 and CD55 on RBCs of AIHA patients and healthy individuals were not statistically different (CD47 = 464.4 and 464.4; CD35 = 186.8 and 194.3; CD55 = 396.9 and 381.1, respectively). Four patients with life-threatening AIHA were treated with high dose of steroids and RBC transfusions, but 3 patients evolved to death. Two patients who died presented low CD55 expression on their RBCs at diagnosis. AIHA patients showed significant lower CD59 expression on RBCs than healthy controls (MFI = 512.3 ± 28.0 and 553.7 ± 36.6, P = .03). Although CD59 expression in patients that evolved to remission was not significantly different from healthy controls (MFI = 538.5 ± 14.4 and 553.7 ± 36.6), the expression of CD59 on RBCs of 3 AHAI patients who died were significantly lower than that seen on RBCs of healthy controls (MFI = 433.6 ± 69.6 and 553.7 ± 36.6, P = .0001). Although experimental studies have suggested that CD47 has a profound influence on the severity of AIHA in mice, our preliminary data on 12 patients with AIHA did not demonstrate difference on the expression of CD47 on RBCs of patients with warm AIHA or healthy indibiduals. On the other hand, complement regulatory proteins (CD35, CD55, and CD59) may play an important role in protecting RBC destruction through the activation of complement. Our results suggest that patients with life-threatening warm AIHA may present significant CD59 deficiency on their RBCs that may increase the susceptibility of cells to complement-mediated lysis resulting in severe clinical hemolysis.

The Serology and the Prognosis of 128 Cases of Autoimmune Hemolytic Anemia

Blood ◽  
1959 ◽  
Vol 14 (12) ◽  
pp. 1280-1301 ◽  
Author(s):  
JEAN DAUSSET ◽  
JACQUES COLOMBANI
Keyword(s):  
Hemolytic Anemia ◽  
Lupus Erythematosus ◽  
Autoimmune Hemolytic Anemia ◽  
Early Stage ◽  
Complete Recovery ◽  
Clinical Results ◽  
Red Cells ◽  
Coombs Test ◽  
Long Term Effect ◽  
Corticosteroid Hormones

Abstract A statistical study of 128 cases of autoimmune hemolytic anemias, serologically followed up in the same laboratory, led to some conclusions on classification, prognosis and treatment. Five forms were distinguished: 1. Idiopathic autoimmune hemolytic anemia with warm autoantibodies (IAHA-wa) was the most frequent form (65 per cent of the cases). It was observed in all peroids of life. A slight predominance among females was noted. This form was characterized clinically by a generalized or conjunctival icterus and a moderate splenomegaly. Hematologically a macrocytic normochromic anemia was present and serologically warm incomplete autoagglutinins, often nonspecific, or sometimes specific for a group antigen, were detected. Hemolysins were not found. The average course was 13 months followed by recovery (54 per cent) and 16 months followed by death (46 per cent). These two groups of patients were compared extensively. No differences in the age, sex, blood group and severity of the initial anemia were noted. A low reticulocyte count, leukopenia and association with thrombocytopenic purpura were more frequent in fatal cases. Tile persistence of a positive indirect Coombs test was unfavorable. Those with free antibodies in the plasma were the most serious. Fifty-two per cent of fatal cases had a positive indirect Coombs test. Of those who recovered, 18.5 per cent had this serologic finding. Transfusions were usually done at the begining of the disease. The efficacy of corticosteroid hormones was confirmed; the percentage of recoveries has risen since this therapy has been used fully (37.5 to 70 per cent). Early or late splenectomy had no influence on final desensitization (long-term effect), but led in 58 per cent of the cases to good clinical results (immediate effect). The spleen destroys red cells coated with noncomplement-fixing antibodies, so that splenectomy leads to compensation for the anemia. One must also describe the acute autoimmune hemolytic anemia observed especially in children, in which warm hemolysins could be detected at the very early stage of the disease. Complement was diminished or absent and the serum often showed anticomplementary activity. Complete recovery was rapid. 2. Symptomatic autoimmune hemolytic anemia with warm autoantibodies (SAHA-wa) accompanied mostly malignant conditions of the lymphocytic or reticuloendothelial systems as well as more rarely disseminated lupus erythematosus (17.6 per cent of the cases). Except for the causal disease, these cases were not different from IAHA-wa and their prognosis depended on the prognosis of the causal disorder. 3. Idiopathic autoimmune hemolytic anemia with cold antibodies (IAHAca) was less frequent (7.7 per cent of the cases). Clinically it was characterized by the rarity of splenomegaly, the chance of cold paroxysmal hemoglobinuria (1 case out of 10) and of Raynaud’s syndrome (1 case out of 10), and serologically by the presence of a cold acid-hemolysin (7 cases out of 8) along with an increased titer of complete agglutinins. Complement was diminished or absent. A positive Coombs test was possibly due to complement fixation. The course of these forms seemed to be very chronic: Nine cases of the 10 of the series were in progress for an average of 26 months, without any apparent trend to densensitization. The action of hormone therapy was less striking than in the warm variety. Splenectomy was probably not effective (1 case), since the red cells sensitized by complement-fixing antibodies were mainly recovered by the liver. 4. Symptomatic autoimmune hemolytic anemia wiith cold antibodies (SAHA-ca) was divided into two distinct forms: (a) one symptomatic of a malignant condition of the blood of the same type as in SAHA-wa (7 per cent of cases). The serology was identical to that of IAHA-ca. The prognosis was determined by that of the causal disease; (b) one symptomatic of a virus or a presumed virus infection (3.9 per cent of cases). Here an acid-hemolysin usually accompanied a very high complete cold agglutinin titer. Complete recovery occurred rapidly. In all cases with cold antibodies exposure to cold had to be carefully avoided. In cases of hemolysins, washed red cells had to be used for transfusions.

P10: IBRUTINIB ASSOCIATED AUTOIMMUNE HEMOLYTIC ANEMIA

2016 ◽  
Vol 64 (3) ◽  
pp. 821.1-821 ◽  
Author(s):  
S Datla ◽  
P Draksharam ◽  
G Sidhu
Keyword(s):  
High Risk ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Kinase Inhibitor ◽  
Lymphocytic Leukemia ◽  
Prior History ◽  
High Dose ◽  
History Of ◽  
Review Reports ◽  
Positive Direct

Purpose of StudyAutoimmune hemolytic anemia (AIHA) is a common phenomenon in Chronic lymphocytic Leukemia (CLL)/Small Lymphocytic Lymphoma (SLL) accounting for about 4–7% of cases. AIHA is commonly associated with certain conventional chemotherapy agents used in CLL/SLL. Ibrutinib, bruton tyrosine kinase inhibitor is category 1 indication for high risk (del 17p) and relapsed/refractory CLL. Literature review reports 11cases of Ibrutinib associated AIHA.We report a case of AIHA precipitated by Ibrutinib in an high risk CLL patient, with prior history of AIHA.Methods UsedPatient is an 81 year old black man diagnosed with asymptomatic Stage I CLL (del 17p) in 2010 and was on active surveillance. He developed AIHA in 2012, with good response to steroids and Rituximab. Subsequently he received 8 cycles of rituximab for symptomatic CLL with resolution of symptoms. In 9/2014, noted to have progression of disease with worsening B symptoms, leukocytosis and lymphadenopathy. He was started on Ibrutinib 420 mg PO daily with regression of lymphadenopathy within 3 weeks of therapy, but presented with symptomatic anemia with hemoglobin of 3 gm/dl, positive direct Coomb's test, elevated reticulocyte count and LDH consistent with AIHA. WBC elevated at 360 K/uL from baseline of 150 K/uL and hemoglobin fell to 3 g/dl from 10 g/dl since Ibrutinib was initiated. Ibrutinib was held and patient received high dose prednisone followed by IVIG and cautious transfusion with minimal improvement in hemoglobin. Hemoglobin slowly up trended with weekly Rituximab and high dose steroids and remained stable around 10 gm/dl after 4 weeks of Rituximab. Ibrutinib was subsequently restarted with overall clinical improvement.Summary of ResultsIn our patient, occurrence of AIHA falls in between 2–4 weeks as other reported cases suggesting that Ibrutinib could be a likely precipitating factor.ConclusionsReview of data, reveals that 22% of patients had history of AIC prior to Ibrutinib, however occurrence of AIHA on Ibrutinib seems to be less common (0.7%).Mechanism of action of Ibrutinib associated cytopenias remains unclear. It was hypothesized that it may be due to IL-2 induced kinase inhibition by Ibrutinib, and needs further investigation.

Autoimmune Hemolytic Anemia in HIV: A Case Series and Review of the Literature.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3858-3858 ◽  
Author(s):  
David P.H. Wu ◽  
Joseph Caperna ◽  
Erin Reid
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Case Series ◽  
High Dose ◽  
Hiv Patients ◽  
Latino Male ◽  
Positive Direct

Abstract Background: Patients infected with HIV are more likely to have positive direct antiglobulin tests (Toy, Am J of Hem 1985) but there have only been a few reports of HIV-related AIHA (autoimmune hemolytic anemia) in the literature. Here we report three new cases of AIHA in HIV infected patients diagnosed from July 2003 to September 2005 at UCSD. Methods: Patients were identified by clinical presentation to the HIV service. All cases were considered highly likely by hematologists to have AIHA (lab data table 1). Results: Patient #1 was a 43 yo Caucasian male with CD4 < 50 prior ITP and untreated recently diagnosed visceral KS. On presentation, he reported dyspnea and had hepatosplenomegaly. His Hb was 5.2, retic 14%, LDH 459, Ibili 0.7, haptoglobin <7; + microspherocytes on smear. He was started on high-dose glucocorticoids (GC) before PRBC transfusion, and tolerated it well other than one fever to 100F. He received prednisone (pred) 60mg daily with taper. 3 weeks later at follow-up he self-tapered pred to 20mg and Hb was 11.1. Once off pred, his Hb remained stable at 11 when he died from PML approximately 4 months after remission of AIHA. Patient #2 is a 43 yo Latino male presenting with syncope, dizziness, dyspnea and palpations. His Hb was 5.4, retic 8%, LDH 366, Ibili 1.2, haptoglobin < 7, with smears showing extensive microspherocytes. He was treated with high-dose GC and received PRBC with heparin prophylaxis. His Hb recovered to 12. He was tapered off GC over 8 months and remains in remission for the past 2 months with Hb 14. Patient #3 is a 38 yo Latino male with dyspnea. His Hb was 5.7, retic 8%. He symptomatically improved with PRBC, and subsequently had a rapid response to GC. Hb peaked at 13.5 through tapering of pred by 10 mg weekly to 30 mg daily. The patient missed follow-up and continued the rapid taper, hemolysis recurred 1 month after pred cessation. Pred 60mg qd was restarted with resolution of hemolysis; this recurred even with slow taper (2mg/week) below 20mg daily. Pred again increased to 60 mg daily and danazol 600mg bid added with resolution of hemolysis. Pred taper to 20 mg was tolerated. Conclusions: AIHA in HIV patients has been described mainly in single case reports and abstracts (Koduri, Am Jo Hematology 2002). Only a few reports include long-term follow-up and fewer have included precipitants for AIHA. Within 2 years, we have three HIV patients with documented AIHA under different clinical manifestations and responses to treatment. One patient had a recent diagnosis of KS, the 2nd had received penicillin for syphilis and the 3rd had no recent medical complications. All 3 patients tolerated transfusion which resulted in symptomatic improvement and responded successfully to pred. Patient #3 who had a high CD4, was the only one with recurrence of hemolysis upon pred taper; perhaps a higher CD4 count may be associated with more persistent AIHA. In the literature, there are 14 other cases of idiopathic AIHA in adults with HIV (summary table to be presented at meeting). Of the 6 cases with reported CD4 counts, all were less than 200. 4/14 cases had inadequate reticulocyte response demonstrating existence of additional factors contributing to anemia. Most cases (10/14) responded to standard AIHA treatments including GC, IVIG and splenectomy. Overall 5/14 had died at the time of the reports; 2 of these deaths were related to PCP after remission of AIHA. 2/8 cases receiving PRBC died of DIC that occurred after transfusion. Table 1 Case Hb gm/dl Retic % LDH IU/L Hapto mg/dl IBili mg/dl CD4 #/ml 1 5.2 13.9 459 <7 0.9 82 2 5.4 8.1 366 <7 1.2 86 3 5.7 8.1 317 <7 2.1 424

scholarly journals An Unusual Case of Autoimmune Hemolytic Anemia-Thinking Outside the Box for Management

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5075-5075
Author(s):  
Paul C Williams ◽  
Ileana Lopez-Plaza
Keyword(s):  
Red Blood Cells ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Blood Cells ◽  
High Dose ◽  
Quadrant Pain ◽  
Lower Quadrant ◽  
Packed Red Blood Cells ◽  
Splenic Embolization ◽  
Positive Direct

Abstract A 27 year old male with no significant past medical history presented to an outside hospital for chief complaints of nausea/vomiting, epigastric pain, and acute jaundice. Initial laboratory workup revealed a hemoglobin of 5.5 g/dl, normal WBC and platelet count, elevated direct bilirubin (10 mg/dl), elevated lactate dehydrogenase (969 IU/L), low haptoglobin (<8 mg/dl), a peripheral smear showing rare polychromasia and spherocytes, and positive direct antiglobulin test (DAT: BS +, IgG +, C3d -) with presence of warm autoantibodies. All infectious disease testing was negative. Initial CT abdomen showed a normal sized spleen. The patient was diagnosed with idiopathic warm autoimmune hemolytic anemia and was treated with pulsed steroids, one dose of Rituximab, and transfused with up to 13 units of packed red blood cells. The patient did not respond to medical treatment, and after one week, the patient was transferred to our institution for escalation of care. At our institution, the patient presented with similar symptoms. Due to lack of response to packed red blood cell transfusions, As the patient continued a downtrend in the hemoglobin despite continued transfusion support, Transfusion Medicine advised to only transfuse phenotypically matched, least incompatible, packed red blood cells for significantly symptomatic anemia or hemodynamic instability. High dose steroid were continued and IVIG, Cytoxan and Vincristine were added to his warm autoimmune hemolytic anemia (WAIHA) therapy. Despite these efforts, the patient's hemoglobin continued to decrease despite an additional 9 RBC units transfused at our institution, reaching a hemoglobin nadir of of 2.1 g/dL. In addition, the patient developed thrombocytopenia, NSTEMI, and left lower quadrant pain accompanied by bloody bowel movements. A repeat CT scan of the abdomen was performed, which revealed an enlarged spleen with a span of 16.5 cm in its maximum dimension. Due to concerns for splenic infarction and/or rupture, and since the patient was not a candidate for surgery, partial splenic artery embolization was performed. Thirty percent residual viable spleen remained. Within hours post embolization, a dramatic increase in hemoglobin was observed without any additional transfusion support. Shortly after splenic embolization the patient developed severe left upper quadrant abdominal pain with a peri-splenic fluid measuring slightly greater than simple fluid attenuation on CT which was managed medically. After close observation the patient was discharged home 2 weeks after admission. At home the patient was doing well with the exception of persistent left upper quadrant pain. He had no clinical or laboratory signs of recurrent hemolysis. Three months post discharge, due to persistent left upper quadrant pain and presence of a splenic cyst, the patient underwent a laparoscopic splenectomy that was performed without complications. During this hospitalization, in preparation for surgery, compatibility testing showed no autoantibodies. However new clinically significant alloantibodies were identified. Hypersplenism and/or reticulocytopenia are infrequent complications of a warm autoimmune hemolytic anemia, constituting a life threatening complication that may not respond to standard therapy and may not qualify for surgical intervention. The above patient did not improve with any medical therapies and displayed a decreasing trend in hemoglobin that was accompanied by increasing lactate levels and NSTEMI. Splenic embolization achieved a dramatic response that was maintained through follow up. Due to recurrent pain from the embolization, the patient eventually received a splenectomy once he was stable. Four months after initial presentation, post infusion of 4 doses of Rituxan and steroid therapy, the autoantibodies were not detectable. However, the patient developed 3 alloantibodies post multiple transfusions. Figure. Figure. Disclosures No relevant conflicts of interest to declare.

scholarly journals Management of Autoimmune Hemolytic Anemia in the Midst of Coronavirus Disease 2019 Pandemic: A Case Report

Medicinus ◽  
2020 ◽  
Vol 7 (7) ◽  
pp. 223
Author(s):  
Andree Kurniawan ◽  
Devina Adella Halim
Keyword(s):  
Body Weight ◽  
Case Report ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immunosuppressive Drugs ◽  
High Dose ◽  
Cell Surface Antigens ◽  
Indirect Bilirubin ◽  
Life Threatening ◽  
Novel Coronavirus

<p><strong>Introduction : </strong>The novel coronavirus disease 2019 (COVID-19) has become a pandemic involving all people and can be severe and life-threatening in a certain population such as those with comorbidity. Autoimmune hemolytic anemia (AIHA) is an autoimmune hematologic disease characterized with antibodies production that binds to red cell surface antigens. In this pandemic, several concerns have been raised by autoimmune disease clinicians and patients regarding the use of immunosuppressive drugs. In this case report, we illustrate the problems of autoimmune hemolytic anemia patient when she got active case.</p><p><strong>Case Illustration : </strong>A 28 years old lady was admitted to the hospital owing to fatigue and tiredness during exercise for two weeks. She had been diagnosed with autoimmune hemolytic anemia before and did not comply with the treatment. This patient has been reevaluated of having AIHA from the symptoms of fatigue, enlarged spleen, low hemoglobin, increased reticulocytes, signs of hemolytic in blood smear examination, increased indirect bilirubin, LDH and the Combs’ test result was given positive. She received methylprednisolone 2 mg/kg of body weight intravenously, washed packed red cells (PRC), calcium and proton pump inhibitor. She was discharged at the seventh day since admission and she was prescribed oral methylprednisolone equal to 1 mg/kg body weight.</p><p><strong>Conclusion : </strong>This is an educated case of non-compliance of AIHA that should be given high dose steroid and blood transfusion during hospitalization amid the COVID-19 pandemic. The recommendation of treatment for AIHA was still the same as before the pandemic occured.</p>

scholarly journals Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature

2021 ◽  
Vol 10 (15) ◽  
pp. 3439
Author(s):  
Irene Motta ◽  
Juri Giannotta ◽  
Marta Ferraresi ◽  
Kordelia Barbullushi ◽  
Nicoletta Revelli ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Case Series ◽  
Intravenous Immunoglobulins ◽  
Point Of View ◽  
First Line ◽  
Human Erythropoietin ◽  
Immune Mediated ◽  
Hemolytic Crisis ◽  
Life Threatening

Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.

scholarly journals NUDT15 polymorphism explains serious toxicity to azathioprine in Indian patients with chronic immune thrombocytopenia and autoimmune hemolytic anemia: a case series

2020 ◽  
Vol 0 (0) ◽  
Author(s):  
Anup J. Devasia ◽  
Raveen Stephen Stallon Illangeswaran ◽  
Infencia Xavier Raj ◽  
Biju George ◽  
Poonkuzhali Balasubramanian
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Immune Thrombocytopenia ◽  
Case Series ◽  
Severe Toxicity ◽  
Toxic Side Effect ◽  
Case Presentation ◽  
Oral Ulcers ◽  
Asian Patients ◽  
Life Threatening

AbstractObjectivesAzathioprine (AZA) is a commonly used immunosuppressant in patients with autoimmune diseases. The toxic side effect to AZA (myelosuppression, hair loss, and oral ulcers) are highly unpredictable which can be life threatening if not identified earlier and dose adjustments made or the drug is withdrawn.Case presentationHere we report a case series of five patients with severe toxicity while on treatment with AZA for autoimmune hemolytic anemia (n=1) and Immune thrombocytopenia (n=4). The common thiopurine methyltransferase (TPMT) variants (TPMT*2, *3A, *3B) were not present in these patients. However, all these patients had the NUDT15 415C>T variant that has been reported to explain serious toxicity to thioguanine in Asian patients.ConclusionsOur report suggests pre-emptive genotype-based dosing of AZA could reduce adverse toxicity and hence better outcome.

scholarly journals Alemtuzumab Plus Cyclosporine Treatment of the Autoimmune Hemolytic Anemia in an Adult Bowel Transplant

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
A. Lauro ◽  
M. Stanzani ◽  
C. Finelli ◽  
C. Zanfi ◽  
M. C. Morelli ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hemolytic Anemia ◽  
Bone Marrow Biopsy ◽  
Autoimmune Hemolytic Anemia ◽  
Leukocyte Count ◽  
Pure Red Cell Aplasia ◽  
Febrile Episode ◽  
High Dose ◽  
Laboratory Findings ◽  
Transfusion Requirements

An adult male underwent a bowel transplant for tufting enteropathy, receiving alemtuzumab, tacrolimus, and steroids as immunosuppressants. Five years later, he developed an autoimmune hemolytic anemia (AIHA), anti-IgG positive, with reduced reticulocyte count, leukopenia, and thrombocytopenia with antiplatelet antibodies. After an unsuccessful initial treatment with high dose steroids, reduction in tacrolimus dose, and intravenous immunoglobulin (IVIG), a bone marrow biopsy revealed absence of erythroid maturation with precursor hyperplasia. The patient was switched to sirolimus and received four doses of rituximab plus two courses of plasmapheresis, which decreased his transfusion requirements. After a febrile episode one month later, the AIHA relapsed with corresponding decreases in platelet and leukocyte count: cyclosporine A (CsA) was started with a second course of rituximab and IVIG without response, even though repeat bone marrow biopsy did not reveal morphology correlated to an acquired pure red cell aplasia (APRCA). Considering the similarity in his clinical and laboratory findings to APRCA, alemtuzumab was added (three doses over a week) with CsA followed by steroids. The patient was eventually discharged transfusion-independent, with increasing hemoglobin (Hb) levels and normal platelet and leukocyte count. One year later he is still disease-free with functioning graft.

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