scholarly journals Hemophagocytic Lymphohistiocytosis in Children

Doctor Ru ◽  
2021 ◽  
Vol 20 (3) ◽  
pp. 66-69
Author(s):  
N.G. Bagdasaryan ◽  
◽  
G.S. Ayvazyan ◽  
L.A. Avetisyan ◽  
N.R. Mnatsakanyan ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Complete Remission ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Case ◽  
Severe Pneumonia ◽  
Bone Marrow Smear ◽  
Cytostatic Agents ◽  
Key Points ◽  
Diagnostic Importance ◽  
Iv Immunoglobulin

Objective of the Paper: To present a clinical case of a 2.5-year girl hospitalised with severe pneumonia with associated hemophagocytic lymphohistiocytosis (HLH). Key Points. HLH is a syndrome, the clinical representation of which is a system sepsis-like inflammatory reaction; in the majority of cases, this condition is hardly diagnosed by paediatricians. Associated pancytopenia and hyperferritinaemia are of diagnostic importance. Here we present a clinical case of a 2.5-year girl who was hospitalised with severe pneumonia and associated HLH. As there is no common approach to the management of HLH, we summoned a board of doctors and decided to start steroid therapy (dexamethasone) and IV immunoglobulin (Octagam), and if there was no effect from therapy within a week, cytostatic agents would be added. The therapy was initiated promptly after macrophages had been found in bone marrow smear. Clinical and paraclinic improvements were noted on day 5, so aggressive immunosuppresion was not initiated. In a month there were no signs of organ dysfunction; the values normalised, and the child was discharged from the inpatient clinic. Conclusion. HLH is associated with high mortality, therefore, early diagnosis and therapy are critical. The specificity of this case is that we managed to promptly diagnose the condition, initiated treatment and achieved complete remission without aggressive immunosuppresion. Keywords: hemophagocytic lymphohistiocytosis, macrophages, pancytopenia.

scholarly journals Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: a single-center study of 22 cases

Blood ◽  
1991 ◽  
Vol 78 (1) ◽  
pp. 51-54 ◽  
Author(s):  
S Blanche ◽  
M Caniglia ◽  
D Girault ◽  
J Landman ◽  
C Griscelli ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Complete Remission ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Marrow Transplantation ◽  
Allogeneic Bone Marrow Transplantation ◽  
Long Term Results ◽  
Intrathecal Methotrexate ◽  
Allogeneic Bone

Abstract Twenty-two children with hemophagocytic lymphohistiocytosis were treated with a chemotherapy regimen consisting of VP16–213, corticosteroids, and intrathecal methotrexate. A sustained clinical and biologic complete remission was obtained in 15 children and a partial remission in one child; six children died early of opportunistic infection (n = 4) or of disease progression (n = 2). Of the 16 children who were placed in first remission, 10 received maintenance chemotherapy alone, while six underwent bone marrow transplantation (HLA matched in five, HLA mismatched in one). Of the children who received chemotherapy alone, only two are in long-term remission after cessation of treatment. The remaining eight patients relapsed after a mean period of 5.4 months (range 2 to 8 months). Further treatment using the same regimen induced second remissions of short duration; death occurred after a median period of 2.3 months (range 0.5 to 6 months). A total of nine patients received allogeneic bone marrow transplantation (BMT). Among the six children transplanted in remission, four are in long-term unmaintained remission, 1 to 6 years after HLA-matched BMT. However, the relapse that occurred in one patient 1 year post BMT is difficult to interpret because the donor, the patient's 5-year-old sister, also developed the disease 1 year later. An HLA-nonidentical BMT resulted in unmaintained remission for 1 year, with autologous hematologic reconstitution followed by disease relapse. HLA-nonidentical BMT failed in three other patients with active disease at time of transplant. The poor long-term results of chemotherapy alone justify the use of related HLA-matched BMT in complete remission.

scholarly journals Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: a single-center study of 22 cases

Blood ◽  
1991 ◽  
Vol 78 (1) ◽  
pp. 51-54 ◽  
Author(s):  
S Blanche ◽  
M Caniglia ◽  
D Girault ◽  
J Landman ◽  
C Griscelli ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Transplantation ◽  
Complete Remission ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Marrow Transplantation ◽  
Allogeneic Bone Marrow Transplantation ◽  
Long Term Results ◽  
Intrathecal Methotrexate ◽  
Allogeneic Bone

Twenty-two children with hemophagocytic lymphohistiocytosis were treated with a chemotherapy regimen consisting of VP16–213, corticosteroids, and intrathecal methotrexate. A sustained clinical and biologic complete remission was obtained in 15 children and a partial remission in one child; six children died early of opportunistic infection (n = 4) or of disease progression (n = 2). Of the 16 children who were placed in first remission, 10 received maintenance chemotherapy alone, while six underwent bone marrow transplantation (HLA matched in five, HLA mismatched in one). Of the children who received chemotherapy alone, only two are in long-term remission after cessation of treatment. The remaining eight patients relapsed after a mean period of 5.4 months (range 2 to 8 months). Further treatment using the same regimen induced second remissions of short duration; death occurred after a median period of 2.3 months (range 0.5 to 6 months). A total of nine patients received allogeneic bone marrow transplantation (BMT). Among the six children transplanted in remission, four are in long-term unmaintained remission, 1 to 6 years after HLA-matched BMT. However, the relapse that occurred in one patient 1 year post BMT is difficult to interpret because the donor, the patient's 5-year-old sister, also developed the disease 1 year later. An HLA-nonidentical BMT resulted in unmaintained remission for 1 year, with autologous hematologic reconstitution followed by disease relapse. HLA-nonidentical BMT failed in three other patients with active disease at time of transplant. The poor long-term results of chemotherapy alone justify the use of related HLA-matched BMT in complete remission.

Intensive care of a complicated form of asthmatic status (clinical case)

2019 ◽  
Vol 2 (31) ◽  
pp. 46-51
Author(s):  
D. A. Temerov ◽  
L. V. Vorobyova ◽  
S. V. Vyzhevsky ◽  
S. B. Savchenkov ◽  
Yu. V. Marchenkov
Keyword(s):  
Clinical Case ◽  
Mixture Distribution ◽  
Severe Pneumonia ◽  
Control Mode ◽  
Respiratory Support ◽  
Oxygen Mixture ◽  
Bronchial Obstruction ◽  
Optimal Regime ◽  
Pulmonary Barotrauma ◽  
Airway Patency

The article presents a clinical case of successful treatment of a patient with prolonged asthmatic status. The pathogenesis of purulent-septic and other life-threatening complications developing as a result of the above critical condition is described. The positive effect of a differentiated approach in conducting respiratory support depending on the stage of the disease is justified: at the beginning, when airway obstruction is in the foreground, and in the future, when restrictive respiratory disorders develop. When conducting respiratory support, the most reasonable methods for ensuring airway patency were selected. The necessity of neurovegetative blockade and myoplegia for the prevention of pulmonary barotrauma during respiratory support by aggressive ventilation modes and with the goal of antihypoxic protection of the brain is emphasized. During the treatment of the patient, it was confirmed that the optimal regime for obstruction of the bronchi is forced volume-cyclic ventilation of the lungs to provide the necessary minute volume of breathing, and in severe pneumonia, in the case of relief of bronchial obstruction, respiratory support is carried out in pressure control mode for better air-oxygen mixture distribution in the airways. The need for early tracheostomy and daily therapeutic fibrobronchoscopy to ensure airway patency and treat pneumonia has been confirmed.

Early Molecular Response of Marrow Disease to Biologic Therapy Is Highly Prognostic in Neuroblastoma

2003 ◽  
Vol 21 (20) ◽  
pp. 3853-3858 ◽  
Author(s):  
Irene Y. Cheung ◽  
M. Serena Lo Piccolo ◽  
Brian H. Kushner ◽  
Nai-Kong V. Cheung
Keyword(s):  
Bone Marrow ◽  
Complete Remission ◽  
Minimal Residual Disease ◽  
Partial Remission ◽  
Progressive Disease ◽  
Residual Disease ◽  
Gm Csf ◽  
Stage 4 ◽  
Secondary Refractory ◽  
Minimal Residual

Purpose: A promising treatment strategy for stage 4 neuroblastoma patients is the repeated application of anti-GD2 immunotherapy after activating myeloid effectors with granulocyte-macrophage colony-stimulating factor (GM-CSF). To use early marrow response as a prognostic marker is particularly relevant for patients not likely to benefit from this therapy. Patients and Methods: Eighty-six stage 4 neuroblastoma patients older than 1 year at diagnosis were classified in four clinical groups on protocol entry: complete remission or very good partial remission (n = 33), primary refractory (n = 33), secondary refractory (n = 10), and progressive disease (n = 10). Bone marrow samples collected before and following treatment were assayed for GD2 synthase mRNA by real-time reverse transcriptase polymerase chain reaction. Response and survival analyses were performed on posttreatment samples before the third cycle at 1.8 months from protocol entry. Results: GD2 synthase mRNA was evident in pretreatment marrow samples of the four clinical groups (42%, 52%, 60%, and 80% of samples, respectively), with median transcript level of 10.0, 16.6, 26.5, and 87.2, respectively. This marker became negative following antibody plus GM-CSF in 77% of complete remission or very good partial remission, 45% of primary refractory, 25% of secondary refractory, and 0% of progressive disease group. Progression-free survival was statistically different between responder and nonresponder groups (P < .0001). Among patients with minimal residual disease, molecular responders had a significantly lower risk of disease progression at a median follow-up of 29.8 months (P = .0001). Conclusion: GD2 synthase mRNA is a sensitive response marker of neuroblastoma in the bone marrow. It is particularly useful for minimal residual disease evaluation and may potentially be useful as an early predictor of resistance to antibody plus GM-CSF immunotherapy.

scholarly journals Hemophagocytic Lymphohistiocytosis Complicating Erythroleukemia in a Child with Monosomy 7: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Samin Alavi ◽  
Maryam Ebadi ◽  
Alireza Jenabzadeh ◽  
M. T. Arzanian ◽  
Sh. Shamsian
Keyword(s):  
Bone Marrow ◽  
Case Report ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Chromosomal Abnormalities ◽  
Bone Marrow Aspiration ◽  
Review Of The Literature ◽  
Monosomy 7 ◽  
Persistent Fever ◽  
First Case ◽  
Erythroid Precursors

Herein, the first case of childhood erythrophagocytosis following chemotherapy for erythroleukemia in a child with monosomy 7 is reported. A 5-year-old boy presented with anemia, thrombocytopenia, and hepatosplenomegaly in whom erythroleukemia was diagnosed. Prolonged pancytopenia accompanied by persistent fever and huge splenomegaly and hepatomegaly became evident after 2 courses of chemotherapy. On bone marrow aspiration, macrophages phagocytosing erythroid precursors were observed and the diagnosis of HLH was established; additionally, monosomy 7 was detected on bone marrow cytogenetic examination. In conclusion, monosomy 7 can lead to erythrophagocytosis associated with erythroid leukemia and should be considered among the chromosomal abnormalities contributing to the association.

Outcome of Allogeneic Hematopoietic Stem-Cell Transplantation in Adult Patients With Acute Lymphoblastic Leukemia: No Difference in Related Compared With Unrelated Transplant in First Complete Remission

2004 ◽  
Vol 22 (14) ◽  
pp. 2816-2825 ◽  
Author(s):  
Michael G. Kiehl ◽  
Ludwig Kraut ◽  
Rainer Schwerdtfeger ◽  
Bernd Hertenstein ◽  
Mats Remberger ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Stem Cell ◽  
Acute Lymphoblastic Leukemia ◽  
Complete Remission ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Lymphoblastic Leukemia ◽  
Philadelphia Chromosome ◽  
Hematopoietic Stem ◽  
First Complete Remission

Purpose The role of unrelated allogeneic stem-cell transplantation in acute lymphoblastic leukemia (ALL) patients is still not clear, and only limited data are available from the literature. We analyzed factors affecting clinical outcome of ALL patients receiving a related or unrelated stem-cell graft from matched donors. Patients and Methods The total study population was 264 adult patients receiving a myeloablative allogeneic stem-cell transplant for ALL at nine bone marrow transplantation centers between 1990 and 2002. Of these, 221 patients receiving a matched related or unrelated graft were analyzed. One hundred forty-eight patients received transplantation in complete remission; 62 patients were in relapse; and 11 patients were refractory to chemotherapy before transplant. Fifty percent of patients received bone marrow, and 50% received peripheral blood stem cell from a human leukocyte antigen–identical related (n = 103), or matched unrelated (n = 118) donor. Results Disease-free survival (DFS) at 5 years was 28%, with 76 patients (34%) still alive (2.2 to 103 months post-transplantation), and 145 deceased (65 relapses, transplant-related mortality, 45%). We observed an advantage regarding DFS in favor of patients receiving transplantation during their first complete remission (CR) in comparison with patients receiving transplantation in or after second CR (P = .014) or who relapsed (P < .001). We observed a clear trend toward improved survival in favor of B-lineage ALL patients compared with T-lineage ALL patients (P = .052), and Philadelphia chromosome–positive patients had no poorer outcome than Philadelphia chromosome–negative patients. Total-body irradiation–based conditioning improved DFS in comparison with busulfan (P = .041). Conclusion Myeloablative matched related or matched unrelated allogeneic hematopoietic stem-cell transplantation in ALL patients should be performed in first CR.

scholarly journals AML cells are differentially sensitive to chemotherapy treatment in a human xenograft model

Blood ◽  
2013 ◽  
Vol 121 (12) ◽  
pp. e90-e97 ◽  
Author(s):  
Mark Wunderlich ◽  
Benjamin Mizukawa ◽  
Fu-Sheng Chou ◽  
Christina Sexton ◽  
Mahesh Shrestha ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Induction Therapy ◽  
Bone Marrow Cells ◽  
Xenograft Model ◽  
Chemotherapy Treatment ◽  
Key Points ◽  
Increased Sensitivity ◽  
Total Bone Marrow ◽  
Marrow Cells

Key Points A relevant xenograft chemotherapy model was developed by using standard AML induction therapy drugs and primary human AML patient samples. Human AML cells show significantly increased sensitivity to in vivo chemotherapy treatment compared with murine LSK and total bone marrow cells.

Secondary Hemophagocytic Lymphohistiocytosis Causing Hemorrhagic Encephalomyelitis and Spinal Subarachnoid Hemorrhage: A Case Report

2021 ◽  
pp. 194187442110446
Author(s):  
Matthew R. Woodward ◽  
Margaret S. Ferris ◽  
Guillermo Rivell ◽  
Laura Malone ◽  
Tara M. Dutta ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Bone Marrow ◽  
Case Report ◽  
Subarachnoid Hemorrhage ◽  
Bone Marrow Biopsy ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Altered Mental Status ◽  
Clinical Context ◽  
Spinal Subarachnoid Hemorrhage ◽  
Secondary Hemophagocytic Lymphohistiocytosis

We are writing to present an interesting and novel case from our practice of a patient who presented with altered mental status and a rapidly progressive paraplegia as well as high fevers and pancytopenia. A bone marrow biopsy was diagnostic of hemophagocytic lymphohistiocytosis (HLH) and MRI showed hemorrhagic encephalitis and spinal subarachnoid hemorrhage. This case demonstrates the diverse neurological symptoms with which HLH presents, including spinal cord pathology. The astute neurologist should consider this diagnosis in the appropriate clinical context and diagnosis may require imaging to the complete neuraxis.

Reticular dysgenesis exacerbated by hemophagocytic lymphohistiocytosis and the presence of unusual histiocyte-like cells in bone marrow

Immunobiology ◽  
2021 ◽  
Vol 226 (6) ◽  
pp. 152143
Author(s):  
Madhubala Sharma ◽  
Rahul Tyagi ◽  
Sathish Kumar Loganathan ◽  
Sreejesh Sreedharaunni ◽  
Amit Rawat ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hemophagocytic Lymphohistiocytosis
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