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HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis

https://doi.org/10.1002/pbc.21039
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52/52 checkable references clean · checked 2026-07-23

Every reference with a DOI in the deposited reference list resolved to a known work in Crossref or DataCite at the dated check, and none carried a retraction, withdrawal, or removal notice.

7 without a DOI — not checked. A reference deposited without a DOI is never matched by title or guessed at; it stays outside the checked set, and this line discloses that.

The 52 checked references that resolve
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FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
resolves10.1016/S0889-8588(05)70521-9
INFECTION- AND MALIGNANCY-ASSOCIATED HEMOPHAGOCYTIC SYNDROMES
resolves10.1111/j.1651-2227.1991.tb11878.x
Incidence in Sweden and Clinical Features of Familial Hemophagocytic Lymphohistiocytosis
resolves10.1007/BF00443367
Familial hemophagocytic lymphohistiocytosis
resolves10.1111/j.1651-2227.1993.tb12699.x
Familial hemophagocytic lymphohistiocytosis and viral infections
resolves10.1002/1097-0142(197909)44:3<993::AID-CNCR2820440329>3.0.CO;2-5
Virus-associated hemophagocytic syndromeA benign histiocytic proliferation distinct from malignant histiocytosis
resolves10.1002/(SICI)1096-911X(199705)28:5<342::AID-MPO3>3.0.CO;2-H
HLH-94: A treatment protocol for hemophagocytic lymphohistiocytosis
resolves10.1111/j.1651-2227.1989.tb11068.x
Hyperferritinemia in Malignant Histiocytosis, Virus‐Associated Hemophagocytic Syndrome and Familial Erythrophagocytic Lymphohistiocytosis
resolves10.1111/j.1651-2227.1991.tb11849.x
Familial Hemophagocytic Lymphohistiocytosis: <i>Clinical Review Based on the Findings in Seven Children</i>
resolves10.1182/blood-2002-04-1030
Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carryingPRF1 mutations
resolves10.1016/S0140-6736(78)91028-0
IMMUNODEFICIENCY IN FAMILIAL ERYTHROPHAGOCYTIC LYMPHOHISTIOCYTOSIS
resolves10.1016/S0022-3476(84)80549-1
Impaired natural killer activity in lymphohistiocytosis syndrome
resolves10.1203/00006450-199810000-00001
Defective Natural Killer Cell Function in Patients with Hemophagocytic Lymphohistiocytosis and in First Degree Relatives
resolves10.1182/blood.V99.1.61
Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family members
resolves10.1182/blood-2001-12-0260
Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell–induced apoptosis
resolves10.1182/blood.V73.8.2128.2128
Elevated soluble interleukin-2 receptor in childhood hemophagocytic histiocytic syndromes
resolves10.1182/blood.V78.11.2918.2918
Hypercytokinemia in familial hemophagocytic lymphohistiocytosis
resolves10.1182/blood.V97.4.1131
Hemophagocytic lymphohistiocytosis due to germline mutations inSH2D1A, the X-linked lymphoproliferative disease gene
resolves10.2174/1566524023362339
Chediak-Higashi Syndrome: a Clinical and Molecular View of a Rare Lysosomal Storage Disorder
resolves10.1023/B:JOCI.0000029119.83799.cb
Griscelli Syndrome: Characterization of a New Mutation and Rescue of T-Cytotoxic Activity by Retroviral Transfer of RAB27A Gene
resolves10.1016/S0022-3476(99)70423-3
Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance
resolves10.1038/sj.bmt.1703396
Allogeneic bone marrow transplantation for active Epstein–Barr virus-related lymphoproliferative disease and hemophagocytic lymphohistiocytosis in an infant with severe combined immunodeficiency syndrome
resolves10.1007/s004310100816
Reviewing Omenn syndrome
resolves10.1093/rheumatology/40.11.1285
Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients
resolves10.1016/0140-6736(92)91008-V
Cerebromeningeal haemophagocytic lymphohistiocytosis
resolves10.1046/j.1365-2141.2003.04726.x
Modern management of children with haemophagocytic lymphohistiocytosis
resolves10.1016/j.critrevonc.2004.01.002
Histiocyte disorders
resolves10.1111/j.1365-2141.2005.05502.x
Subtyping of natural killer cell cytotoxicity deficiencies in haemophagocytic lymphohistocytosis provides therapeutic guidance
resolves10.1046/j.1365-2141.1999.01538.x
Induction of apoptosis and caspase activation in cells obtained from familial haemophagocytic lymphohistiocytosis patients
resolves10.1126/science.286.5446.1957
Perforin Gene Defects in Familial Hemophagocytic Lymphohistiocytosis
resolves10.1086/318796
Spectrum of Perforin Gene Mutations in Familial Hemophagocytic Lymphohistiocytosis
resolves10.1136/jmg.2003.011528
Characterisation of diverse <i>PRF1</i> mutations leading to decreased natural killer cell activity in North American families with haemophagocytic lymphohistiocytosis
resolves10.3109/10428190109097672
Familial Hemophagocytic Lymphohistiocytosis: Too Little Cell Death Can Seriously Damage Your Health
resolves10.1016/S0092-8674(03)00855-9
Munc13-4 Is Essential for Cytolytic Granules Fusion and Is Mutated in a Form of Familial Hemophagocytic Lymphohistiocytosis (FHL3)
resolves10.1093/hmg/ddi076
Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11
resolves10.1002/humu.20274
Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses ofPRF1, UNC13D, STX11, andRAB27A
resolves10.1136/jmg.2005.035253
Spectrum and clinical implications of syntaxin 11 gene mutations in familial haemophagocytic lymphohistiocytosis: association with disease-free remissions and haematopoietic malignancies
resolves10.1002/1097-0142(19800515)45:10<2516::AID-CNCR2820451008>3.0.CO;2-V
Successful treatment of lymphohistiocytic reticulosis with phagocytosis with epipodophyllotoxin VP 16–213
resolves10.1016/S0140-6736(86)92047-7
SUCCESSFUL INDUCTION WITH CHEMOTHERAPY INCLUDING TENIPOSIDE IN FAMILIAL ERYTHROPHAGOCYTIC LYMPHOHISTIOCYTOSIS
resolves10.1182/blood.V82.8.2319.2319
Treatment of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins, steroids, and cyclosporin A
resolves10.1182/blood-2002-01-0172
Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation
resolves10.1182/blood.V89.3.794
Frequency and Severity of Central Nervous System Lesions in Hemophagocytic Lymphohistiocytosis
resolves10.1016/S0022-3476(97)70196-3
Neuropathologic findings and neurologic symptoms in twenty-three children with hemophagocytic lymphohistiocytosis
resolves10.1016/S0022-3476(86)81002-2
Allogeneic bone marrow transplantation for erythrophagocytic lymphohistiocytosis
resolves10.1182/blood.V90.12.4743
Treatment of Familial Hemophagocytic Lymphohistiocytosis With Bone Marrow Transplantation From HLA Genetically Nonidentical Donors
resolves10.1046/j.1365-2141.1999.01625.x
Improved outcome in haemophagocytic lymphohistiocytosis after bone marrow transplantation from related and unrelated donors: a single‐centre experience of 12 patients
resolves10.1080/0888001039024301
Clinical and Genetic Studies of Familial Hemophagocytic Lymphohistiocytosis in Oman: Need for Early Treatment
resolves10.1159/000069773
Biochemical and Clinical Aspects of Methotrexate Neurotoxicity
resolves10.1200/JCO.2001.19.10.2665
Requirement for Etoposide in the Treatment of Epstein-Barr Virus–Associated Hemophagocytic Lymphohistiocytosis
resolves10.1111/j.1365-2141.2005.05501.x
Haematopoietic stem cell transplantation in haemophagocytic lymphohistiocytosis
resolves10.1542/peds.2005-1789
Hematopoietic Stem Cell Transplantation in Hemophagocytic Lymphohistiocytosis: A Single-Center Report of 48 Patients
resolves10.1182/blood-2005-05-1819
Stem cell transplantation with reduced-intensity conditioning for hemophagocytic lymphohistiocytosis
The 7 references without a DOI — listed, not checked
no DOI — not checkedDiagnostic guidelines for hemophagocytic lymphohistiocytosis
no DOI — not checkedHemophagocytic lymphohistiocytosis: Diagnosis, treatment and prognostic factors. Report of 122 children from the international registry
no DOI — not checkedHemophagocytic lymphohistiocytosis: A hemophagocytic syndrome
no DOI — not checkedX‐linked lymphoproliferative disease: Genetics and biochemistry
no DOI — not checkedEffective control of Epstein–Barr virus‐related hemophagocytic lymphohistiocytosis with immunochemotherapy
no DOI — not checkedMacrophage activation syndrome is hemophagocytic lymphohistiocytosis—need for the right terminology
no DOI — not checkedTreatment of four patients with erythrophagocytic lymphohistiocytosis by a combination of epipodophyllotoxin, steroids, intrathecal methotrexate and cranial irradiation
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