CLINICAL PRESENTATION AND OUTCOME OF BENIGN HISTIOCYTIC DISORDERS IN CHILDREN
(Abstract release date: 05/19/16)
EHA Library. A Tantawy A. 06/09/16; 132959; E1410
Prof. Dr. Azza A Tantawy
Contributions
Contributions
Abstract
Abstract: E1410
Type: Eposter Presentation
Background
Langerhans cell histiocytosis (LCH)& Hemophagocytic lymphohistiocytosis (HLH) are the most common benign histiocytic disorders. The clinical picture of LCH varies from single-system to multisystem disease which carries a potentially poor prognosis. HLH is referred to as either a“primary” form which is hereditary, or a “secondary” form associated with an underlying illness. The disease is invariably fatal if untreated.
Aims
Our goal was to evaluate the different clinical presentations of histiocytic disorders, mainly LCH and HLH. and to determine their outcome and survival in relation to the predisposing factors and the current treatment protocols
Methods
Demographic, clinical, laboratory and radiographic data of patients diagnosed with LCH and HLH over a period of 10 years was retrosecpectively collected. Protocol of treatment was evaluated including the 1 and 3 year event free and overall survival rates
Results
This study included 19 LCH patients (median age 2.3 years) with a males to female ratio of 1:1.7. All LCH patients had multisystem affection, 16 (84.2%) had risk organs involvement. Seven patients(36.8%) had Bone marrow infiltration; 13 (68.4%) patients had hepatosplenomegaly and 14 (73.7%) had lung infiltrations in their CT. In our study 19(94.7%) of LCH patients received LCH III protocol, while only one (5.3%) patient received LCHII protocol of treatment. Six (31.6%) had received salvage line, half of them (3 patients) needed only one treatment protocol, 1patient needed 3 salvage and the last 2 patients received 2 salvage treatment. Six patients died, the median duration from diagnosis till death duration was 1.9 year. Their 1,3 years overall survival was 78.9% and 46.1%. Twenty six patients with HLH were registered; their median age was 3.4 yrs with a male to female ratio of 2:1. Ten patients had HLH secondary to another disease (2 patients (7.7%) had ALL, 2 (7.7%) had Chediak Hegashi, 3(11.5%) had LCH, one had Wolman, one with myelodyplasia and one with ALPS). Sixteen (61.5%) patients did not have an underlying disease, 4 of them(15.4%) had MUNC13-4 gene mutation, 1(3.8%) had RAB27A gene mutation and 1(3.8%) patient had LYST gene mutation, and 2 patients had no common HlH mutations. Thrombosis was reported in 2(7.7%) of the patients and (19.3%) of them presented initially with skin lesions, moreover five (19.3%) patients had CNS manifestations but only 2 of them had positive findings in their MRI and CT brain. Most of the patients were treated according to HLH 2004 protocol, while only 2 received HLH 94 protocol. twelve patients (46.2%) had reactivations, 6 needed only one reinduction, 4 had 2 reinductions and 2 had 3 reinductions. We report the death of 18 patients, with a median duration since the diagnosis till death of 3.36months.
Conclusion
All our LCH patients had multisystem (MS) risk organ affection, hence the high reactivation and the low overall survival rate. The increased awareness of the HLH criteria for diagnosis, allowed early and accurate detection of cases, yet the death rate is still high.
Session topic: E-poster
Keyword(s): Dendritic cell, Macrophage, Remission, Survival
Type: Eposter Presentation
Background
Langerhans cell histiocytosis (LCH)& Hemophagocytic lymphohistiocytosis (HLH) are the most common benign histiocytic disorders. The clinical picture of LCH varies from single-system to multisystem disease which carries a potentially poor prognosis. HLH is referred to as either a“primary” form which is hereditary, or a “secondary” form associated with an underlying illness. The disease is invariably fatal if untreated.
Aims
Our goal was to evaluate the different clinical presentations of histiocytic disorders, mainly LCH and HLH. and to determine their outcome and survival in relation to the predisposing factors and the current treatment protocols
Methods
Demographic, clinical, laboratory and radiographic data of patients diagnosed with LCH and HLH over a period of 10 years was retrosecpectively collected. Protocol of treatment was evaluated including the 1 and 3 year event free and overall survival rates
Results
This study included 19 LCH patients (median age 2.3 years) with a males to female ratio of 1:1.7. All LCH patients had multisystem affection, 16 (84.2%) had risk organs involvement. Seven patients(36.8%) had Bone marrow infiltration; 13 (68.4%) patients had hepatosplenomegaly and 14 (73.7%) had lung infiltrations in their CT. In our study 19(94.7%) of LCH patients received LCH III protocol, while only one (5.3%) patient received LCHII protocol of treatment. Six (31.6%) had received salvage line, half of them (3 patients) needed only one treatment protocol, 1patient needed 3 salvage and the last 2 patients received 2 salvage treatment. Six patients died, the median duration from diagnosis till death duration was 1.9 year. Their 1,3 years overall survival was 78.9% and 46.1%. Twenty six patients with HLH were registered; their median age was 3.4 yrs with a male to female ratio of 2:1. Ten patients had HLH secondary to another disease (2 patients (7.7%) had ALL, 2 (7.7%) had Chediak Hegashi, 3(11.5%) had LCH, one had Wolman, one with myelodyplasia and one with ALPS). Sixteen (61.5%) patients did not have an underlying disease, 4 of them(15.4%) had MUNC13-4 gene mutation, 1(3.8%) had RAB27A gene mutation and 1(3.8%) patient had LYST gene mutation, and 2 patients had no common HlH mutations. Thrombosis was reported in 2(7.7%) of the patients and (19.3%) of them presented initially with skin lesions, moreover five (19.3%) patients had CNS manifestations but only 2 of them had positive findings in their MRI and CT brain. Most of the patients were treated according to HLH 2004 protocol, while only 2 received HLH 94 protocol. twelve patients (46.2%) had reactivations, 6 needed only one reinduction, 4 had 2 reinductions and 2 had 3 reinductions. We report the death of 18 patients, with a median duration since the diagnosis till death of 3.36months.
Conclusion
All our LCH patients had multisystem (MS) risk organ affection, hence the high reactivation and the low overall survival rate. The increased awareness of the HLH criteria for diagnosis, allowed early and accurate detection of cases, yet the death rate is still high.
Session topic: E-poster
Keyword(s): Dendritic cell, Macrophage, Remission, Survival
Abstract: E1410
Type: Eposter Presentation
Background
Langerhans cell histiocytosis (LCH)& Hemophagocytic lymphohistiocytosis (HLH) are the most common benign histiocytic disorders. The clinical picture of LCH varies from single-system to multisystem disease which carries a potentially poor prognosis. HLH is referred to as either a“primary” form which is hereditary, or a “secondary” form associated with an underlying illness. The disease is invariably fatal if untreated.
Aims
Our goal was to evaluate the different clinical presentations of histiocytic disorders, mainly LCH and HLH. and to determine their outcome and survival in relation to the predisposing factors and the current treatment protocols
Methods
Demographic, clinical, laboratory and radiographic data of patients diagnosed with LCH and HLH over a period of 10 years was retrosecpectively collected. Protocol of treatment was evaluated including the 1 and 3 year event free and overall survival rates
Results
This study included 19 LCH patients (median age 2.3 years) with a males to female ratio of 1:1.7. All LCH patients had multisystem affection, 16 (84.2%) had risk organs involvement. Seven patients(36.8%) had Bone marrow infiltration; 13 (68.4%) patients had hepatosplenomegaly and 14 (73.7%) had lung infiltrations in their CT. In our study 19(94.7%) of LCH patients received LCH III protocol, while only one (5.3%) patient received LCHII protocol of treatment. Six (31.6%) had received salvage line, half of them (3 patients) needed only one treatment protocol, 1patient needed 3 salvage and the last 2 patients received 2 salvage treatment. Six patients died, the median duration from diagnosis till death duration was 1.9 year. Their 1,3 years overall survival was 78.9% and 46.1%. Twenty six patients with HLH were registered; their median age was 3.4 yrs with a male to female ratio of 2:1. Ten patients had HLH secondary to another disease (2 patients (7.7%) had ALL, 2 (7.7%) had Chediak Hegashi, 3(11.5%) had LCH, one had Wolman, one with myelodyplasia and one with ALPS). Sixteen (61.5%) patients did not have an underlying disease, 4 of them(15.4%) had MUNC13-4 gene mutation, 1(3.8%) had RAB27A gene mutation and 1(3.8%) patient had LYST gene mutation, and 2 patients had no common HlH mutations. Thrombosis was reported in 2(7.7%) of the patients and (19.3%) of them presented initially with skin lesions, moreover five (19.3%) patients had CNS manifestations but only 2 of them had positive findings in their MRI and CT brain. Most of the patients were treated according to HLH 2004 protocol, while only 2 received HLH 94 protocol. twelve patients (46.2%) had reactivations, 6 needed only one reinduction, 4 had 2 reinductions and 2 had 3 reinductions. We report the death of 18 patients, with a median duration since the diagnosis till death of 3.36months.
Conclusion
All our LCH patients had multisystem (MS) risk organ affection, hence the high reactivation and the low overall survival rate. The increased awareness of the HLH criteria for diagnosis, allowed early and accurate detection of cases, yet the death rate is still high.
Session topic: E-poster
Keyword(s): Dendritic cell, Macrophage, Remission, Survival
Type: Eposter Presentation
Background
Langerhans cell histiocytosis (LCH)& Hemophagocytic lymphohistiocytosis (HLH) are the most common benign histiocytic disorders. The clinical picture of LCH varies from single-system to multisystem disease which carries a potentially poor prognosis. HLH is referred to as either a“primary” form which is hereditary, or a “secondary” form associated with an underlying illness. The disease is invariably fatal if untreated.
Aims
Our goal was to evaluate the different clinical presentations of histiocytic disorders, mainly LCH and HLH. and to determine their outcome and survival in relation to the predisposing factors and the current treatment protocols
Methods
Demographic, clinical, laboratory and radiographic data of patients diagnosed with LCH and HLH over a period of 10 years was retrosecpectively collected. Protocol of treatment was evaluated including the 1 and 3 year event free and overall survival rates
Results
This study included 19 LCH patients (median age 2.3 years) with a males to female ratio of 1:1.7. All LCH patients had multisystem affection, 16 (84.2%) had risk organs involvement. Seven patients(36.8%) had Bone marrow infiltration; 13 (68.4%) patients had hepatosplenomegaly and 14 (73.7%) had lung infiltrations in their CT. In our study 19(94.7%) of LCH patients received LCH III protocol, while only one (5.3%) patient received LCHII protocol of treatment. Six (31.6%) had received salvage line, half of them (3 patients) needed only one treatment protocol, 1patient needed 3 salvage and the last 2 patients received 2 salvage treatment. Six patients died, the median duration from diagnosis till death duration was 1.9 year. Their 1,3 years overall survival was 78.9% and 46.1%. Twenty six patients with HLH were registered; their median age was 3.4 yrs with a male to female ratio of 2:1. Ten patients had HLH secondary to another disease (2 patients (7.7%) had ALL, 2 (7.7%) had Chediak Hegashi, 3(11.5%) had LCH, one had Wolman, one with myelodyplasia and one with ALPS). Sixteen (61.5%) patients did not have an underlying disease, 4 of them(15.4%) had MUNC13-4 gene mutation, 1(3.8%) had RAB27A gene mutation and 1(3.8%) patient had LYST gene mutation, and 2 patients had no common HlH mutations. Thrombosis was reported in 2(7.7%) of the patients and (19.3%) of them presented initially with skin lesions, moreover five (19.3%) patients had CNS manifestations but only 2 of them had positive findings in their MRI and CT brain. Most of the patients were treated according to HLH 2004 protocol, while only 2 received HLH 94 protocol. twelve patients (46.2%) had reactivations, 6 needed only one reinduction, 4 had 2 reinductions and 2 had 3 reinductions. We report the death of 18 patients, with a median duration since the diagnosis till death of 3.36months.
Conclusion
All our LCH patients had multisystem (MS) risk organ affection, hence the high reactivation and the low overall survival rate. The increased awareness of the HLH criteria for diagnosis, allowed early and accurate detection of cases, yet the death rate is still high.
Session topic: E-poster
Keyword(s): Dendritic cell, Macrophage, Remission, Survival
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