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Hematopoietic and Lymphatic System Disorder

MedGen UID:: 208860
•Concept ID:: C0851353
•: Disease or Syndrome

Synonyms:

Blood and Lymphatic System Disorders; Hemic and Lymphatic Diseases

Definition

A non-neoplastic or neoplastic disorder that affects the hematopoietic and lymphatic system. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVHematopoietic and Lymphatic System Disorder

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Disorder by Site
      - Hematopoietic and Lymphatic System Disorder
        Bone marrow disorder
        Bone marrow failure syndrome
        Aplastic anemia
        Inherited bone marrow failure syndrome
        Bone marrow neoplasm
        Acquired polycythemia vera
        Bone marrow cancer
        Bone Marrow Suppression
        Gelatinous Bone Marrow Transformation
        Myelodysplastic syndrome
        Adult Myelodysplastic Syndrome
        Childhood myelodysplastic syndrome
        de novo Myelodysplastic Syndrome
        Familial monosomy 7 syndrome
        Hereditary Myelodysplastic Syndrome
        Monocytopenia with susceptibility to infections
        Myelodysplastic syndrome associated with isolated del(5q)
        Myelodysplastic Syndrome with del(5q)
        Myelodysplastic syndrome with excess blasts
        Myelodysplastic syndrome with multilineage dysplasia
        Myelodysplastic syndrome with ring sideroblasts
        Myelodysplastic Syndrome with Somatic Mutation
        Myelodysplastic Syndrome, Unclassifiable
        Paroxysmal Hemoglobinuria
        Recurrent Myelodysplastic Syndrome
        Refractory anemia
        Refractory anemia with excess blasts in transformation
        Refractory anemia with ringed sideroblasts
        Refractory cytopenia with unilineage dysplasia
        Refractory Myelodysplastic Syndrome
        Secondary Myelodysplastic Syndrome
        Sideroblastic anemia
        Myelodysplastic/myeloproliferative neoplasm
        Atypical chronic myeloid leukemia, BCR-ABL1 negative
        Chronic myelomonocytic leukemia
        Juvenile myelomonocytic leukemia
        Myelodysplastic/Myeloproliferative Neoplasm Post Cytotoxic Therapy
        Myelodysplastic/Myeloproliferative Neoplasm with Ring Sideroblasts and Thrombocytosis, Not Otherwise Specified
        Recurrent Myelodysplastic/Myeloproliferative Neoplasm
        Refractory Myelodysplastic/Myeloproliferative Neoplasm
        Unclassified myelodysplastic/myeloproliferative disease
        Myelofibrosis
        Myelofibrosis due to another disorder
        Primary myelofibrosis
        Recurrent Myelofibrosis
        Refractory Myelofibrosis
        Myeloproliferative disorder
        Acute myeloid leukemia, M6 type
        Chronic myelogenous leukemia, BCR-ABL1 positive
        Chronic neutrophilic leukemia
        Leukemoid reaction
        Myelophthisic anemia
        Thrombocytosis
        Transient myeloproliferative syndrome
        Pancytopenia
        Aplastic bone marrow
        Pancytopenia caused by medication
        Hematologic disorder
        Abnormality of the coagulation cascade
        Abnormal bleeding
        Abnormality of prothrombin
        Abnormality of the common coagulation pathway
        Abnormality of the extrinsic pathway
        Abnormality of the intrinsic pathway
        Abnormality of the protein C anticoagulant pathway
        Abnormality of von Willebrand factor
        Blood coagulation disorder due to liver disease
        Coagulation factor deficiency syndrome
        Coagulation protein disease
        Congenital Bleeding Disorder
        Congenital plasminogen activator inhibitor type 1 deficiency
        COVID-19-Associated Coagulation Disorder
        Disseminated intravascular coagulation
        Dysfibrinogenemia
        Ecchymosis
        Essential thrombocythemia
        Fibrinolysis Shutdown
        Hemolytic-uremic syndrome
        Hereditary antithrombin deficiency
        High molecular weight kininogen deficiency
        Hyperfibrinogenemia
        Immune-Mediated Coagulopathy
        Inherited blood coagulation disorder
        Plasma Kallikrein Deficiency
        Platelet-type bleeding disorder 17
        Protein S deficiency disease
        Purpura
        Storage pool disease of platelets
        Thromboinflammation
        Thrombophilia
        Thrombotic microangiopathy
        Tissue Kallikrein Deficiency
        Vitamin K deficiency
        von Willebrand disorder
        Anemia
        Anemia due to chronic disorder
        Anemia due to Decreased Production
        Anemia due to Disorder of Glutathione Metabolism
        Anemia due to disorders of nucleotide metabolism
        Anemia due to enzyme disorder
        Anemia due to Increased Destruction
        Anemia due to reduced life span of red cells
        Anemia of inadequate production
        Anemia of prematurity
        Congenital anemia
        Hemolytic anemia
        Hypochromia
        Hypochromic anemia
        Macrocytic anemia
        Microcytic anemia
        Mild Anemia
        Neonatal anemia
        Normocytic anemia
        Pure red-cell aplasia
        Radiation Anemia
        Steroid-responsive anemia
        Transient erythroblastopenia
        Treatment-Induced Anemia
        Blood Protein Disorders
        Agammaglobulinemia
        Dysgammaglobulinemia
        Hypergammaglobulinemia
        Paraproteinemia
        Reduced protein C activity
        Blood transfusion reaction
        Acute lung injury during and following administration of blood product
        Congenital analbuminemia
        Hematologic neoplasm
        Leukemia
        Lymphoma
        Lymphoproliferative disorder
        Multiple myeloma
        Myelodysplasia
        Myeloproliferative disorder, chronic, with eosinophilia
        Plasmacytoma
        Hematopoietic and lymphoid cell neoplasm
        Central nervous system hematopoietic neoplasm
        Cutaneous Hematopoietic and Lymphoid Cell Neoplasm
        Histiocytic and dendritic cell neoplasm
        Lymphoid neoplasm
        Myeloid neoplasm
        Myeloid/lymphoid neoplasms associated with eosinophilia and abnormality of PDGFRA, PDGFRB, FGFR1 or JAK2
        Recurrent Hematologic Malignancy
        Refractory hematologic cancer
        Transplant-Related Hematologic Malignancy
        Hemoglobinopathy
        Hb SS disease
        Hemoglobin C disease
        Hemoglobin D disease
        Hemoglobin E disease
        Hemoglobin Trait
        Sickle cell-beta-thalassemia
        Sickle cell-hemoglobin C disease
        Sickle cell-hemoglobin D disease
        Sickle cell-hemoglobin E disease syndrome
        Sickle cell-Hemoglobin O Arab disease
        Sickle cell-thalassemia disease
        Thalassemia
        Vaso-Occlusive Crisis
        Hemolytic disease of fetus OR newborn due to isoimmunization
        Anti-D Hemolytic Disease of the Newborn
        Bilirubin encephalopathy
        Hemolytic disease of fetus OR newborn due to ABO immunization
        Hydrops fetalis
        Rhesus Hemolytic Disease of the Newborn
        Hemorrhagic disease
        Afibrinogenemia
        Autoimmune thrombocytopenic purpura
        Bernard Soulier syndrome
        Congenital factor V deficiency
        Factor VII deficiency
        Factor X deficiency
        Factor XII deficiency disease
        Glanzmann thrombasthenia
        Hereditary factor IX deficiency disease
        Hereditary factor VIII deficiency disease
        Hereditary factor XI deficiency disease
        Hereditary factor XIII deficiency disease
        Prothrombin deficiency
        Vascular hemostatic disease
        Waterhouse-Friderichsen Syndrome
        Wiskott-Aldrich syndrome
        Inherited bleeding disorder, platelet-type
        Gray platelet syndrome
        Qualitative platelet disorder
        Thrombocytopenia
        L-ferritin deficiency
        Leukocyte disorder
        Eosinophilia
        Infectious mononucleosis
        Leukocytosis
        Leukopenia
        Leukostasis
        Pelger-Huët anomaly
        Phagocyte bactericidal dysfunction
        Methemoglobinemia
        Acquired methemoglobinemia
        Hereditary methemoglobinemia
        Non-Neoplastic Hematopoietic and Lymphoid Cell Disorder
        Amegakaryocytosis
        Benign Lymphoproliferative Disorder
        Congenital hematological disorder
        Cyclical neutropenia
        Evans syndrome
        Functional disorder of polymorphonuclear neutrophil
        Neonatal Leukopenia
        Paroxysmal nocturnal hemoglobinuria
        Sinus histiocytosis with massive lymphadenopathy
        Spherocytosis
        Polyclonal hyperviscosity syndrome
        Polycythemia
        Increased red blood cell mass
        Multiple paragangliomas associated with polycythemia
        Polycythemia (Excluding Polycythemia Vera)
        Primary familial polycythemia due to EPO receptor mutation
        Pregnancy Complications, Hematologic
        Preleukemias
        Premalignant hematological system disease
        Atypical lymphoproliferative disorder
        B-Cell Proliferation of Uncertain Malignant Potential
        Clonal Cytopenia of Undetermined Significance
        Clonal hematopoiesis
        Idiopathic Cytopenia of Undetermined Significance
        Idiopathic Dysplasia of Uncertain Significance
        T-Cell Proliferation of Uncertain Malignant Potential
        Sulfhemoglobinemia
        Thrombocytopenic purpura
        Thrombotic thrombocytopenic purpura
        Twin anemia-polycythemia sequence
        Hematopoietic and lymphoid system neoplasm
        Lymph node neoplasm
        Benign neoplasm of lymph node
        Lymph node cancer
        Spleen neoplasm
        Benign neoplasm of spleen
        Spleen cancer
        Lymph node disorder
        Lymph node abscess
        Lymphadenitis
        Acute lymphadenitis
        Axillary lymphadenitis
        Cat scratch disease
        cervical lymphadenitis
        Chronic lymphadenitis
        Kikuchi-Fujimoto disease
        Kimura disease
        Mesenteric lymphadenitis
        Postauricular lymphadenitis
        Submandibular adenitis
        Suppurative lymphadenitis
        Lymphoid system disorder
        Acute febrile mucocutaneous lymph node syndrome
        Histiocytosis
        Langerhans cell histiocytosis
        Malignant histiocytic disorder
        Mucinous histiocytosis
        Non-Langerhans cell histiocytosis
        Lymphadenopathy
        Angioimmunoblastic T-cell lymphoma
        Atypical Lymph Node
        Axillary lymphadenopathy
        Cervical lymphadenopathy
        Chronic noninfectious lymphadenopathy
        Dermatopathic lymphadenitis
        Follicular hyperplasia
        Generalized lymphadenopathy
        Hilar lymph node enlargement
        Inguinal lymphadenopathy
        Localized enlarged lymph nodes
        Lymphadenopathy with Polyclonal Hypergammaglobulinemia
        Paratracheal lymphadenopathy
        Submandibular lymph node enlargement
        Supraclavicular lymphadenopathy
        Lymphangiectasis
        Congenital pulmonary lymphangiectasia
        Intestinal lymphangiectasia
        Pancreatic lymphangiectasis
        Pericardial lymphangiectasia
        Pleural lymphangiectasia
        Pulmonary lymphangiectasia
        Thyroid lymphangiectasia
        Lymphangitis
        Lymphatic malformation
        22q11 Deletion Syndrome
        Lymphedema
        Breast Cancer Lymphedemas
        Chronic acquired lymphedema
        Elephantiasis
        Filarial elephantiasis
        Hereditary lymphedema type I
        Lymphedema of the Head and Neck
        Non-Filarial Lymphedema
        Predominantly lower limb lymphedema
        Lymphocele
        Renal Lymphocele
        Neoplasm of the thymus
        Benign neoplasm of thymus
        Thymic carcinoid tumor
        Thymic epithelial neoplasm
        Thymic neuroendocrine tumor
        Thymoma
        Thymus cancer
        Thymus Solitary Fibrous Tumor
        Pseudolymphoma
        Cervical Florid Reactive Lymphoid Hyperplasia
        Conjunctival Reactive Lymphoid Hyperplasia
        Reactive lymphoid hyperplasia of lacrimal gland
        Splenic disorder
        Hypersplenism
        Infection of spleen
        Splenic infarction
        Splenic rupture
        Splenic tuberculosis
        Visceral heterotaxy
        Wandering spleen
        Thymus hyperplasia

Professional guidelines

PubMed

Primary central nervous system lymphoma.

Ferreri AJM, Calimeri T, Cwynarski K, Dietrich J, Grommes C, Hoang-Xuan K, Hu LS, Illerhaus G, Nayak L, Ponzoni M, Batchelor TT
Nat Rev Dis Primers 2023 Jun 15;9(1):29. doi: 10.1038/s41572-023-00439-0. PMID: 37322012 Free PMC Article

Diagnosis and Treatment of Chronic Lymphocytic Leukemia: A Review.

Shadman M
JAMA 2023 Mar 21;329(11):918-932. doi: 10.1001/jama.2023.1946. PMID: 36943212

Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management.

Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, Aghamohammadi A
J Investig Allergol Clin Immunol 2020;30(1):14-34. Epub 2019 Feb 11 doi: 10.18176/jiaci.0388. PMID: 30741636

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Recent clinical studies

Etiology

How I prevent and treat central nervous system disease in adults with acute lymphoblastic leukemia.

Kopmar NE, Cassaday RD
Blood 2023 Mar 23;141(12):1379-1388. doi: 10.1182/blood.2022017035. PMID: 36548957 Free PMC Article

Histiocytic Sarcoma.

Tocut M, Vaknine H, Potachenko P, Elias S, Zandman-Goddard G
Isr Med Assoc J 2020 Oct;22(10):645-647. PMID: 33070490

Treatment of relapsed/refractory paediatric aggressive B-cell non-Hodgkin lymphoma.

Moleti ML, Testi AM, Foà R
Br J Haematol 2020 Jun;189(5):826-843. Epub 2020 Mar 6 doi: 10.1111/bjh.16461. PMID: 32141616

Adult acute leukemia.

Cornell RF, Palmer J
Dis Mon 2012 Apr;58(4):219-38. doi: 10.1016/j.disamonth.2012.01.011. PMID: 22449370

Chronic lymphocytic leukemia.

Shanshal M, Haddad RY
Dis Mon 2012 Apr;58(4):153-67. doi: 10.1016/j.disamonth.2012.01.009. PMID: 22449365

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Diagnosis

Chronic active Epstein-Barr virus disease originates from infected hematopoietic stem cells.

Wang J, Su M, Wei N, Yan H, Zhang J, Gong Y, Wu L, Suolitiken D, Pi Y, Song D, Chen L, Liu H, Yang S, Wang X, Wang Z
Blood 2024 Jan 4;143(1):32-41. doi: 10.1182/blood.2023021074. PMID: 37824804

Histiocytosis and the nervous system: from diagnosis to targeted therapies.

Cohen Aubart F, Idbaih A, Emile JF, Amoura Z, Abdel-Wahab O, Durham BH, Haroche J, Diamond EL
Neuro Oncol 2021 Sep 1;23(9):1433-1446. doi: 10.1093/neuonc/noab107. PMID: 33993305 Free PMC Article

Treatment of relapsed/refractory paediatric aggressive B-cell non-Hodgkin lymphoma.

Moleti ML, Testi AM, Foà R
Br J Haematol 2020 Jun;189(5):826-843. Epub 2020 Mar 6 doi: 10.1111/bjh.16461. PMID: 32141616

Adenosine deaminase deficiency: a review.

Flinn AM, Gennery AR
Orphanet J Rare Dis 2018 Apr 24;13(1):65. doi: 10.1186/s13023-018-0807-5. PMID: 29690908 Free PMC Article

Non-Hodgkin Lymphoma: Diagnosis and Treatment.

Ansell SM
Mayo Clin Proc 2015 Aug;90(8):1152-63. doi: 10.1016/j.mayocp.2015.04.025. PMID: 26250731

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Therapy

Management of Philadelphia chromosome positive acute lymphoblastic leukemia in the current era.

Badar T, Alkhateeb H, Aljurf M, Kharfan-Dabaja MA
Curr Res Transl Med 2023 Apr-Jun;71(2):103392. Epub 2023 Apr 23 doi: 10.1016/j.retram.2023.103392. PMID: 37121211

How I treat relapsed acute lymphoblastic leukemia in the pediatric population.

Hunger SP, Raetz EA
Blood 2020 Oct 15;136(16):1803-1812. doi: 10.1182/blood.2019004043. PMID: 32589723

Treatment of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia.

Abou Dalle I, Jabbour E, Short NJ, Ravandi F
Curr Treat Options Oncol 2019 Jan 24;20(1):4. doi: 10.1007/s11864-019-0603-z. PMID: 30675645 Free PMC Article

How I manage patients with grey zone lymphoma.

Kritharis A, Pilichowska M, Evens AM
Br J Haematol 2016 Aug;174(3):345-50. Epub 2016 Jun 15 doi: 10.1111/bjh.14174. PMID: 27301470

Whipple's disease.

Feldman M
Am J Med Sci 1986 Jan;291(1):56-67. doi: 10.1097/00000441-198601000-00011. PMID: 2417481

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Prognosis

Acute lymphoblastic leukemia.

Chang JH, Poppe MM, Hua CH, Marcus KJ, Esiashvili N
Pediatr Blood Cancer 2021 May;68 Suppl 2:e28371. doi: 10.1002/pbc.28371. PMID: 33818880

Treatment of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia.

Abou Dalle I, Jabbour E, Short NJ, Ravandi F
Curr Treat Options Oncol 2019 Jan 24;20(1):4. doi: 10.1007/s11864-019-0603-z. PMID: 30675645 Free PMC Article

Acute Lymphoblastic Leukemia in Children.

Hunger SP, Mullighan CG
N Engl J Med 2015 Oct 15;373(16):1541-52. doi: 10.1056/NEJMra1400972. PMID: 26465987

Familial Hemophagocytic Lymphohistiocytosis.

Degar B
Hematol Oncol Clin North Am 2015 Oct;29(5):903-13. Epub 2015 Aug 25 doi: 10.1016/j.hoc.2015.06.008. PMID: 26461150

Non-Hodgkin Lymphoma: Diagnosis and Treatment.

Ansell SM
Mayo Clin Proc 2015 Aug;90(8):1152-63. doi: 10.1016/j.mayocp.2015.04.025. PMID: 26250731

See all (6408)

Clinical prediction guides

Chronic active Epstein-Barr virus disease originates from infected hematopoietic stem cells.

Wang J, Su M, Wei N, Yan H, Zhang J, Gong Y, Wu L, Suolitiken D, Pi Y, Song D, Chen L, Liu H, Yang S, Wang X, Wang Z
Blood 2024 Jan 4;143(1):32-41. doi: 10.1182/blood.2023021074. PMID: 37824804

Langerhans cell histiocytosis: promises and caveats of targeted therapies in high-risk and CNS disease.

Abla O
Hematology Am Soc Hematol Educ Program 2023 Dec 8;2023(1):386-395. doi: 10.1182/hematology.2023000439. PMID: 38066856 Free PMC Article

Plerixafor injection: a hematopoietic stem cell mobilizer in non-Hodgkin lymphoma and multiple myeloma.

Jantunen E, Varmavuo V, Valtola J
Expert Rev Hematol 2016 Aug;9(8):723-32. Epub 2016 Jul 15 doi: 10.1080/17474086.2016.1208082. PMID: 27355397

Haematopoietic development and leukaemia in Down syndrome.

Roberts I, Izraeli S
Br J Haematol 2014 Dec;167(5):587-99. Epub 2014 Aug 22 doi: 10.1111/bjh.13096. PMID: 25155832

MUM1/IRF4: A Review.

Gualco G, Weiss LM, Bacchi CE
Appl Immunohistochem Mol Morphol 2010 Jul;18(4):301-10. doi: 10.1097/PAI.0b013e3181cf1126. PMID: 20182347

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Recent systematic reviews

Comparison of blinatumomab and CAR T-cell therapy in relapsed/refractory acute lymphoblastic leukemia: a systematic review and meta-analysis.

Zhai Y, Hong J, Wang J, Jiang Y, Wu W, Lv Y, Guo J, Tian L, Sun H, Li Y, Li C, Zhan H, Zhao Z
Expert Rev Hematol 2024 Jan-Mar;17(1-3):67-76. Epub 2024 Jan 4 doi: 10.1080/17474086.2023.2298732. PMID: 38135295

Comparison of Allogeneic Stem Cell Transplant and Autologous Stem Cell Transplant in Refractory or Relapsed Peripheral T-Cell Lymphoma: A Systematic Review and Meta-analysis.

Du J, Yu D, Han X, Zhu L, Huang Z
JAMA Netw Open 2021 May 3;4(5):e219807. doi: 10.1001/jamanetworkopen.2021.9807. PMID: 34042995 Free PMC Article

Chemotherapy for non-Hodgkin lymphoma in the hemodialysis patient: A comprehensive review.

Yasuda H, Yasuda M, Komatsu N
Cancer Sci 2021 Jul;112(7):2607-2624. Epub 2021 Jun 11 doi: 10.1111/cas.14933. PMID: 33938097 Free PMC Article

Burkitt and Burkitt-Like Lymphomas: a Systematic Review.

Saleh K, Michot JM, Camara-Clayette V, Vassetsky Y, Ribrag V
Curr Oncol Rep 2020 Mar 6;22(4):33. doi: 10.1007/s11912-020-0898-8. PMID: 32144513

Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review.

Hayden A, Park S, Giustini D, Lee AY, Chen LY
Blood Rev 2016 Nov;30(6):411-420. Epub 2016 May 20 doi: 10.1016/j.blre.2016.05.001. PMID: 27238576

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