A Case of Rhesus Hemolytic Disease With Hemophagocytosis and Severe Iron Overload Due to Multiple Transfusions

Yılmaz, Şebnem; Duman, Neval; Özer, Erdener; Kavas, Nazan; Ören, Hale; Demircioğlu, Fatih; Kumral, Abdullah; Özkan, Hasan; İrken, Gülersu

doi:10.1097/01.mph.0000212906.07018.93

Cited by 22 publications

(23 citation statements)

References 18 publications

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“…Chelation therapy resulted in rapid reduction in ferritin levels. In the literature, chelation therapy was required for 6-12 weeks [23,24]. In our case, chelation therapy was given for 10 weeks.…”

Section: Discussionmentioning

confidence: 99%

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Severe iron overload and hyporegenerative anemia in a case with rhesus hemolytic disease: therapeutic approach to rare complications

Demircioğlu¹,

Sözmen²,

Yılmaz³

et al. 2010

Turk J Hematol

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A 33 weeks' gestation, a baby with rhesus hemolytic disease (RHD), who had received intrauterine transfusions twice, developed cholestatic hepatic disease and late hyporegenerative anemia. Her serum ferritin and bilirubin levels increased to 8842 ng/ml and 17.9 mg/dl, respectively. Liver biopsy showed cholestasis and severe iron overload. Treatment with recombinant erythropoietin (rHuEPO) decreased the transfusion need, and intravenous deferoxamine resulted in a marked decreased in serum ferritin levels and normalization of liver function. In patients who have undergone intrauterine transfusions due to RHD, hyperferritinemia and late hyporegenerative anemia should be kept in mind. Chelation therapy in cases with symptomatic hyperferritinemia and rHuEPO treatment in cases with severe hyporegenerative anemia should be considered. (Turk J Hematol 2010; 27: 204-8) Key words: Rhesus hemolytic disease, late hyporegenerative anemia, transfusion-related hepatic iron overload, chelation therapy

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Section: Discussionmentioning

confidence: 99%

“…The clinical course of RHD patients with excess iron load is variable; while most cases have no clinical findings, chelation therapy can be necessary in some cases with severe organ damage [22][23][24][25]. Ferritin and hepatic iron content are extremely variable in neonates such that there are no definite criteria for chelation therapy.…”

Section: Discussionmentioning

confidence: 99%

Severe iron overload and hyporegenerative anemia in a case with rhesus hemolytic disease: therapeutic approach to rare complications

Demircioğlu¹,

Sözmen²,

Yılmaz³

et al. 2010

Turk J Hematol

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“…Some of these studies (mostly case reports) describe that cholestasis in neonates with HDN is uncommon and usually mild and transient [3][4][5] . Other reports however detail severe and protracted courses of cholestasis [2,4,6,7] . The etiology of cholestatic liver disease in neonates with HDN has been associated with iron overload due to intrauterine transfusions (IUTs) [6,[8][9][10] .…”

Section: Introductionmentioning

confidence: 99%

Cholestasis in Neonates with Red Cell Alloimmune Hemolytic Disease: Incidence, Risk Factors and Outcome

Smits-Wintjens¹,

Rath²,

Lindenburg³

et al. 2012

Neonatology

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Background: Etiology of cholestatic liver disease in neonates with hemolytic disease of the newborn (HDN) has been associated with iron overload due to intrauterine red cell transfusions (IUTs). Data on the incidence and severity of cholestasis in neonates with HDN are scarce, and little is known about pathogenesis, risk factors, neonatal management and outcome. Objective: To evaluate incidence, risk factors, management and outcome of cholestasis in neonates with red cell alloimmune hemolytic disease. Methods: All (near-) term neonates with HDN due to red cell alloimmunization admitted to our center between January 2000 and July 2010 were included in this observational study. Liver function tests (including conjugated bilirubin) were routinely performed in the neonatal period. We recorded the presence of cholestasis, investigated several potential risk factors and evaluated the management and outcome in affected neonates. Results: A total of 313 infants with red cell alloimmune hemolytic disease treated with or without IUTs were included. The incidence of cholestasis was 13% (41/313). Two risk factors were independently associated with cholestasis: treatment with at least one IUT (OR 5.81, 95% CI 1.70–19.80, p = 0.005) and rhesus D type of alloimmunization (OR 4.66, 95% CI 1.05–20.57, p = 0.042). Additional diagnostic tests to investigate possible causes of cholestasis were all negative. In 5 infants (12%), supportive medical and nutritional therapy was started, and one neonate required iron chelation therapy. Conclusion: Cholestasis occurs in 13% of neonates with HDN due to red cell alloimmunization, and it is independently associated with IUT treatment and rhesus D type of alloimmunization.

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“…10,11 Data on HLH associated with neonatal cholestasis are limited in the literature. Yılmaz et al 12 performed exchange transfusions twice, in the 31st and 32nd gestational weeks, in a newborn who had ascites and conjugated hyperbilirubinemia at parturition and received blood transfusions twice due to Rh disease and severe anemia. Intravenous immunoglobulin (IVIG, 1 g/kg) was administered upon diagnosis of HLH on day 2.…”

Section: Discussionmentioning

confidence: 99%

“…As seen in Yılmaz's case, multiorgan failure, autoimmune diseases, congenital hemolytic anemia, blood transfusion, and hypofibrinogenemia may trigger the secondary HLH. 12 The primary objective in HLH treatment is to suppress hypercytokinemia, which is responsible for the occurrence of devastating symptoms. The HLH-2004 protocol, a standardized treatment, includes the combined use of etoposide cyclosporine A, and corticosteroid (available at URL: www.histio.org/society/protocols).…”

Section: Discussionmentioning

confidence: 99%

Haemophagocytic Lymphohistiocytosis in a Newborn Infant Presenting with Cholestasis: Case Report

Kahveci¹

2012

UHOD

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, fatal disease. Neonatal cholestasis exhibits symptoms similar to those seen in several newborn diseases. HLH is rapidly fatal; therefore, an effective and prompt differential diagnosis is vital. A 10-hour-old newborn with icterus was referred to our clinic. Laboratory examination revealed direct bilirubinemia and pancytopenia, and cholestasis developed. HLH findings were observed in the bone marrow aspiration. Induction chemotherapy as described in the HLH-2004 protocol was initiated. Despite notable improvement in clinical signs and laboratory findings, the infant died, probably due to sepsis, one week after start of chemotherapy HLH should be kept in mind in the differential diagnosis of all cholestatic patients with recently developed cytopenia. For definitive diagnosis of HLH, clinical signs and laboratory findings of the patient should be evaluated, hyperferritinemia and hypertriglyceridemia should be searched and bone marrow aspiration materials should be examined carefully. Keywords: Hemophagocytic lymphohistiocytosis, Cholestasis, Infant ÖZET Kolestazla Baflvuran Yenidoan ‹nfantta Hemofagositik Lenfohistiositoz: Olgu SunumuHemofagositik lenfohistiositozis (HLH) nadir ve ölümcül bir hastal›kt›r. Neonatal kolestazis birçok ciddi yenido¤an hastal›¤›nda görü-len ortak bir bulgudur. HLH h›zla ölümcül olabilece¤inden etkili ve h›zl› tan› konulmas› hayati öneme sahiptir. Klini¤imize 10 saatlik, sar›l›¤› olan bir yenido¤an sevk edildi. Laboratuar tetkiklerinde direkt bilirubinemi, pansitopeni ve kolestaz varl›¤› saptand›. Yap›lan kemik ili¤i aspirasyonunda HLH bulgular› gözlendi. HLH-2004 protokolünde belirtildi¤i flekilde indüksiyon kemoterapisi baflland›. Klinik ve laboratuar bulgularda görülen iyileflmeye ra¤men hastam›z kemoterapi bafllad›ktan bir hafta sonra olas› sepsis sebebiyle kaybedildi. HLH, sitopeni geliflen tüm kolestatik hastalar›n ay›r›c› tan›s›nda ak›lda tutulmas› gereken bir hastal›kt›r. HLH'nin kesin tan›s›n› koyabilmek için hastan›n klinik ve laboratuar bulgular› de¤erlendirilmeli, hiperferritinemi ve hipertrigliseridemi araflt›r›lmal› ve kemik ili¤i aspirasyon materyali dikkatlice incelenmelidir.

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A Case of Rhesus Hemolytic Disease With Hemophagocytosis and Severe Iron Overload Due to Multiple Transfusions

Abstract: We suggest that patients who have undergone IUT be evaluated for hyperferritinemia. If hyperferritinemia is noted, chelation therapy should be considered. As another rare finding, HLH can complicate the course of RHD.

Cited by 22 publications

References 18 publications

Severe iron overload and hyporegenerative anemia in a case with rhesus hemolytic disease: therapeutic approach to rare complications

Severe iron overload and hyporegenerative anemia in a case with rhesus hemolytic disease: therapeutic approach to rare complications

Cholestasis in Neonates with Red Cell Alloimmune Hemolytic Disease: Incidence, Risk Factors and Outcome

Haemophagocytic Lymphohistiocytosis in a Newborn Infant Presenting with Cholestasis: Case Report

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