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Non-Neoplastic Hematopoietic and Lymphoid Cell Disorder

MedGen UID:: 276575
•Concept ID:: C1518374
•: Disease or Syndrome

Synonyms:

Non-Malignant Hematologic and Lymphocytic Disorder; Non-Neoplastic Hematologic and Lymphocytic Disorder; Non-Neoplastic Hematologic and Lymphoid Cell Disorder

Definition

A non-neoplastic disorder that arises from hematopoietic and lymphoid cells. It is characterized by the absence of a clonal cellular component. It rarely progresses to a malignant neoplasm. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVNon-Neoplastic Hematopoietic and Lymphoid Cell Disorder

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Disorder by Site
      - Hematopoietic and Lymphatic System Disorder
        Hematologic disorder
        Non-Neoplastic Hematopoietic and Lymphoid Cell Disorder
        Abnormality of the coagulation cascade
        Abnormal bleeding
        Abnormal onset of bleeding
        Abnormal umbilical stump bleeding
        Bleeding requiring red cell transfusion
        Bleeding with minor or no trauma
        Cephalohematoma
        Epistaxis
        Excessive bleeding after a venipuncture
        Excessive bleeding from superficial cuts
        Gingival bleeding
        Menorrhagia
        Persistent bleeding after trauma
        Prolonged bleeding following procedure
        Prolonged bleeding time
        Abnormality of prothrombin
        Abnormal prothrombin time
        Reduced prothrombin antigen
        Reduced prothrombin consumption
        Abnormality of the common coagulation pathway
        Abnormal coagulation factor V activity
        Abnormality of circulating fibrinogen
        Decreased level of heparin co-factor II
        Decreased level of histidine-rich glycoprotein
        Decreased level of thrombomodulin
        Decreased level of tissue plasminogen activator
        Increased plasma vitamin K epoxide after vitamin K supplementation
        Prolonged Russell viper venom time
        Reduced alpha-2-antiplasmin activity
        Reduced antithrombin antigen
        Reduced factor X activity
        Reduced factor XIII activity
        Abnormality of the extrinsic pathway
        Reduced factor VII activity
        Abnormality of the intrinsic pathway
        Abnormal factor IX activity
        Abnormal factor VIII activity
        Factor XII deficiency disease
        Hereditary antithrombin deficiency
        High molecular weight kininogen deficiency
        Reduced factor XI activity
        Abnormality of the protein C anticoagulant pathway
        Reduced protein C activity
        Reduced protein S activity
        Resistance to activated protein C
        Abnormality of von Willebrand factor
        Abnormal von Willebrand factor multimer distribution
        Enhanced ristocetin cofactor assay activity
        Impaired binding of factor VIII to VWF
        Impaired ristocetin cofactor assay activity
        Impaired ristocetin-induced platelet aggregation
        Impaired von Willebrand factor collagen binding activity
        Increased ratio of VWF propeptide to VWF antigen
        Reduced quantity of Von Willebrand factor
        Reduced von Willebrand factor activity
        Blood coagulation disorder due to liver disease
        Coagulation factor deficiency syndrome
        Acquired coagulation factor deficiency
        Factor V deficiency
        Factor VII deficiency
        Factor X deficiency
        Factor XIII deficiency
        Fibrinogen deficiency
        Hemophilia
        Hereditary coagulation factor deficiency
        Prothrombin deficiency
        Tissue Factor Deficiency
        Coagulation protein disease
        Afibrinogenemia
        Congenital factor V deficiency
        Hereditary factor IX deficiency disease
        Hereditary factor VIII deficiency disease
        Hereditary factor XI deficiency disease
        Hereditary factor XIII deficiency disease
        von Willebrand disorder
        Congenital Bleeding Disorder
        Congenital plasminogen activator inhibitor type 1 deficiency
        COVID-19-Associated Coagulation Disorder
        COVID-19-Associated Thromboembolism
        Disseminated intravascular coagulation
        Acute disseminated intravascular coagulation
        Chronic disseminated intravascular coagulation
        Disseminated intravascular coagulation in newborn
        Dysfibrinogenemia
        Ecchymosis
        Battle sign
        Conjunctival Ecchymosis
        Ecchymosis of gingivae
        Essential thrombocythemia
        Thrombocythemia 1
        Thrombocythemia 2
        Thrombocythemia 3
        Fibrinolysis Shutdown
        Hemolytic-uremic syndrome
        Atypical hemolytic-uremic syndrome
        Atypical hemolytic-uremic syndrome with DGKE deficiency
        Cobalamin C disease
        D-plus hemolytic uremic syndrome (D+HUS)
        Diarrhea-associated hemolytic uremic syndrome
        Diarrhea-negative hemolytic uremic syndrome
        Infection-related hemolytic uremic syndrome
        Hyperfibrinogenemia
        Immune-Mediated Coagulopathy
        Inherited blood coagulation disorder
        Bernard Soulier syndrome
        Glanzmann thrombasthenia
        Gray platelet syndrome
        Hermansky-Pudlak syndrome
        Upshaw-Schulman syndrome
        Wiskott-Aldrich syndrome
        Plasma Kallikrein Deficiency
        Platelet-type bleeding disorder 17
        Protein S deficiency disease
        Purpura
        Hyperglobulinemic purpura
        Immunoglobulin A vasculitis
        Macular purpura
        Palpable purpura
        Purpura fulminans
        Thrombocytopenic purpura
        Vascular purpura
        Waterhouse-Friderichsen Syndrome
        Storage pool disease of platelets
        Thromboinflammation
        Thrombophilia
        Hereditary thrombophilia
        Thrombotic thrombocytopenic purpura
        Thrombotic microangiopathy
        Systemic lupus erythematosus
        Transplant-Associated Thrombotic Microangiopathy
        Tissue Kallikrein Deficiency
        Vitamin K deficiency
        Vitamin K Deficiency Bleeding
        Amegakaryocytosis
        Congenital Amegakaryocytosis
        Non-Congenital Amegakaryocytosis
        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
        Uridine monophosphate hydrolase deficiency
        Anemia due to enzyme disorder
        Anemia due to erythrocyte enzyme disorder
        Anemia due to Increased Destruction
        Anemia due to reduced life span of red cells
        Hemolytic anemia
        Increased red cell sickling tendency
        Anemia of inadequate production
        Hypochromic anemia
        Hypoplastic anemia
        Macrocytic anemia
        Microcytic anemia
        Normochromic anemia
        Normocytic anemia
        Sideroblastic anemia
        Anemia of prematurity
        Aplastic anemia
        Aplastic anemia due to drugs
        Aplastic anemia due to infection
        Aplastic anemia due to radiation
        Hypoplastic anemia - familial
        Idiopathic aplastic anemia
        Non-Severe Aplastic Anemia
        Recurrent Aplastic Anemia
        Severe Aplastic Anemia
        Very Severe Aplastic Anemia
        Congenital anemia
        Congenital dyserythropoietic anemia
        Familial hemolytic anemia
        Hereditary sideroblastic anemia
        Hypochromia
        Mild Anemia
        Myelophthisic anemia
        Neonatal anemia
        Fetal-maternal hemorrhage
        Twin-to-twin transfusion
        Pure red-cell aplasia
        Acquired red cell aplasia
        Diamond-Blackfan anemia
        Radiation Anemia
        Refractory anemia
        Myelodysplastic syndrome with excess blasts
        Steroid-responsive anemia
        Transient erythroblastopenia
        Treatment-Induced Anemia
        Benign Lymphoproliferative Disorder
        Chronic lymphoproliferative disorder of NK-cells
        Follicular Bronchitis/Bronchiolitis
        Infectious mononucleosis
        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
        Lymphocytoma cutis
        Congenital hematological disorder
        Beta-thalassemia-X-linked thrombocytopenia syndrome
        Congenital Abnormalities of Erythrocyte Differentiation or Function
        Congenital neutropenia
        Severe congenital neutropenia
        Congenital Platelets Abnormality
        Genetic anomaly of leukocyte
        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
        Hereditary methemoglobinemia
        Deficiency of cytochrome-b5 reductase
        Methemoglobin reductase deficiency
        Methemoglobinemia type 4
        Thrombocytopenia 1
        Thrombocytopenia, X-linked, with or without dyserythropoietic anemia
        Cyclical neutropenia
        Cyclic neutropenia in myeloid maturation arrest in bone marrow
        Evans syndrome
        Functional disorder of polymorphonuclear neutrophil
        Chronic granulomatous disease
        Granulomatous disease, chronic, autosomal recessive, cytochrome b-negative
        Granulomatous disease, chronic, autosomal recessive, cytochrome b-positive, type 1
        Granulomatous disease, chronic, autosomal recessive, cytochrome b-positive, type 2
        Granulomatous disease, chronic, autosomal recessive, cytochrome b-positive, type 3
        Granulomatous disease, chronic, X-linked
        Chédiak-Higashi syndrome
        Aleutian disease
        Leukocyte adhesion deficiency
        Leukocyte adhesion deficiency 1
        Leukocyte adhesion deficiency 3
        Leukocyte adhesion deficiency type II
        Pelger-Huët anomaly
        Inherited bleeding disorder, platelet-type
        Qualitative platelet disorder
        Thrombocytopenia
        11q partial monosomy syndrome
        Amegakaryocytic thrombocytopenia
        Congenital thrombocytopenia
        Heparin-induced thrombocytopenia
        Intermittent thrombocytopenia
        Kasabach-Merritt syndrome
        Neonatal alloimmune thrombocytopenia
        Post-transfusion thrombocytopenia
        Thrombocytosis
        Familial thrombocytosis
        Methemoglobinemia
        Acquired methemoglobinemia
        Drug-induced methemoglobinemia
        Toxic methemoglobinemia with cyanosis
        Neonatal Leukopenia
        Paroxysmal nocturnal hemoglobinuria
        Sinus histiocytosis with massive lymphadenopathy
        Bone Rosai-Dorfman-Destombes Disease
        Cutaneous Rosai-Dorfman-Destombes Disease
        Massive Sinusoidal Histiocytic Infiltrate
        Thyroid Gland Rosai-Dorfman-Destombes Disease
        Spherocytosis
        Hereditary spherocytosis
        Hereditary spherocytosis type 1
        Hereditary spherocytosis type 2
        Hereditary spherocytosis type 3
        Hereditary spherocytosis type 4
        Hereditary spherocytosis type 5
        Microspherocytosis

Professional guidelines

PubMed

PAX-5: a valuable immunohistochemical marker in the differential diagnosis of lymphoid neoplasms.

Desouki MM, Post GR, Cherry D, Lazarchick J
Clin Med Res 2010 Jul;8(2):84-8. doi: 10.3121/cmr.2010.891. PMID: 20660931 Free PMC Article

See all (1)

Recent clinical studies

Etiology

How predictive is the finding of clonal hematopoiesis for the development of myelodysplastic syndromes (MDS) or acute myeloid leukemia (AML)?

Steensma DP
Best Pract Res Clin Haematol 2021 Dec;34(4):101327. Epub 2021 Oct 23 doi: 10.1016/j.beha.2021.101327. PMID: 34865699

Fatal donor-derived Kaposi sarcoma following liver transplantation.

Copeland MMM, Trainor J, Cash WJ, Braniff C
BMJ Case Rep 2021 Jun 22;14(6) doi: 10.1136/bcr-2020-236061. PMID: 34158319 Free PMC Article

Multiparameter flow cytometry is instrumental to distinguish myelodysplastic syndromes from non-neoplastic cytopenias.

Cremers EMP, Westers TM, Alhan C, Cali C, Wondergem MJ, Poddighe PJ, Ossenkoppele GJ, van de Loosdrecht AA
Eur J Cancer 2016 Feb;54:49-56. Epub 2015 Dec 22 doi: 10.1016/j.ejca.2015.11.013. PMID: 26720403

Particulate cytoplasmic structures with high concentration of ubiquitin-proteasome accumulate in myeloid neoplasms.

Pecci A, Necchi V, Barozzi S, Vitali A, Boveri E, Elena C, Bernasconi P, Noris P, Solcia E
J Hematol Oncol 2015 Jun 18;8:71. doi: 10.1186/s13045-015-0169-6. PMID: 26081257 Free PMC Article

Mesenchymal tumors of the uterine corpus with heterologous and hematopoietic components: a study of ten cases and review of the literature.

Kondi-Pafiti A, Grapsa D, Kairi-Vassilatou E, Kontogianni-Katsarou K, Koliopoulos C, Botsis D
Eur J Gynaecol Oncol 2006;27(1):73-7. PMID: 16550975

See all (9)

Diagnosis

Multiparameter flow cytometry is instrumental to distinguish myelodysplastic syndromes from non-neoplastic cytopenias.

Autoimmune myelofibrosis: an update on morphologic features in 29 cases and review of the literature.

Vergara-Lluri ME, Piatek CI, Pullarkat V, Siddiqi IN, O'Connell C, Feinstein DI, Brynes RK
Hum Pathol 2014 Nov;45(11):2183-91. Epub 2014 Aug 13 doi: 10.1016/j.humpath.2014.07.017. PMID: 25282037

PAX-5: a valuable immunohistochemical marker in the differential diagnosis of lymphoid neoplasms.

Desouki MM, Post GR, Cherry D, Lazarchick J
Clin Med Res 2010 Jul;8(2):84-8. doi: 10.3121/cmr.2010.891. PMID: 20660931 Free PMC Article

Mesenchymal tumors of the uterine corpus with heterologous and hematopoietic components: a study of ten cases and review of the literature.

Kondi-Pafiti A, Grapsa D, Kairi-Vassilatou E, Kontogianni-Katsarou K, Koliopoulos C, Botsis D
Eur J Gynaecol Oncol 2006;27(1):73-7. PMID: 16550975

Oligoclonal non-neoplastic B cell expansion is the key feature of type II mixed cryoglobulinemia: clinical and molecular findings do not support a bone marrow pathologic diagnosis of indolent B cell lymphoma.

De Vita S, De Re V, Gasparotto D, Ballarè M, Pivetta B, Ferraccioli G, Pileri S, Boiocchi M, Monteverde A
Arthritis Rheum 2000 Jan;43(1):94-102. doi: 10.1002/1529-0131(200001)43:1<94::AID-ANR12>3.0.CO;2-8. PMID: 10643704

See all (13)

Therapy

Fatal donor-derived Kaposi sarcoma following liver transplantation.

Copeland MMM, Trainor J, Cash WJ, Braniff C
BMJ Case Rep 2021 Jun 22;14(6) doi: 10.1136/bcr-2020-236061. PMID: 34158319 Free PMC Article

HLA-C and HLA-DQB1 compatibility in unrelated cord blood transplants.

Elia L, Arcese W, Torello M, Iori AP, Guglielmi C, Perrone MP, Screnci M, Sprovieri T, Rapanotti MC, Cimino G
Haematologica 1999 Jun;84(6):530-4. PMID: 10366797

Detection and distribution of hepatitis C virus-related proteins in lymph nodes of patients with type II mixed cryoglobulinemia and neoplastic or non-neoplastic lymphoproliferation.

Sansonno D, De Vita S, Cornacchiulo V, Carbone A, Boiocchi M, Dammacco F
Blood 1996 Dec 15;88(12):4638-45. PMID: 8977256

Recent progress in multiple myeloma.

Moscinski LC, Ballester OF
Hematol Oncol 1994 May-Jun;12(3):111-23. doi: 10.1002/hon.2900120303. PMID: 7525447

See all (4)

Prognosis

How predictive is the finding of clonal hematopoiesis for the development of myelodysplastic syndromes (MDS) or acute myeloid leukemia (AML)?

Steensma DP
Best Pract Res Clin Haematol 2021 Dec;34(4):101327. Epub 2021 Oct 23 doi: 10.1016/j.beha.2021.101327. PMID: 34865699

Multiparameter flow cytometry is instrumental to distinguish myelodysplastic syndromes from non-neoplastic cytopenias.

Autoimmune myelofibrosis: an update on morphologic features in 29 cases and review of the literature.

Vergara-Lluri ME, Piatek CI, Pullarkat V, Siddiqi IN, O'Connell C, Feinstein DI, Brynes RK
Hum Pathol 2014 Nov;45(11):2183-91. Epub 2014 Aug 13 doi: 10.1016/j.humpath.2014.07.017. PMID: 25282037

Mesenchymal tumors of the uterine corpus with heterologous and hematopoietic components: a study of ten cases and review of the literature.

Kondi-Pafiti A, Grapsa D, Kairi-Vassilatou E, Kontogianni-Katsarou K, Koliopoulos C, Botsis D
Eur J Gynaecol Oncol 2006;27(1):73-7. PMID: 16550975

Recent progress in multiple myeloma.

Moscinski LC, Ballester OF
Hematol Oncol 1994 May-Jun;12(3):111-23. doi: 10.1002/hon.2900120303. PMID: 7525447

See all (7)

Clinical prediction guides

How predictive is the finding of clonal hematopoiesis for the development of myelodysplastic syndromes (MDS) or acute myeloid leukemia (AML)?

Steensma DP
Best Pract Res Clin Haematol 2021 Dec;34(4):101327. Epub 2021 Oct 23 doi: 10.1016/j.beha.2021.101327. PMID: 34865699

Comparative High-Resolution Transcriptome Sequencing of Lymphoma Cell Lines and de novo Lymphomas Reveals Cell-Line-Specific Pathway Dysregulation.

Taher L, Beck J, Liu W, Roolf C, Soller JT, Rütgen BC, Hammer SE, Chodisetti M, Sender S, Sterenczak KA, Fuellen G, Junghanss C, Brenig B, Nolte I, Schütz E, Murua Escobar H
Sci Rep 2018 Apr 19;8(1):6279. doi: 10.1038/s41598-018-23207-7. PMID: 29674676 Free PMC Article

Multiparameter flow cytometry is instrumental to distinguish myelodysplastic syndromes from non-neoplastic cytopenias.

Particulate cytoplasmic structures with high concentration of ubiquitin-proteasome accumulate in myeloid neoplasms.

Pecci A, Necchi V, Barozzi S, Vitali A, Boveri E, Elena C, Bernasconi P, Noris P, Solcia E
J Hematol Oncol 2015 Jun 18;8:71. doi: 10.1186/s13045-015-0169-6. PMID: 26081257 Free PMC Article

Myeloperoxidase: a specific marker for myeloid cells in paraffin sections.

Pinkus GS, Pinkus JL
Mod Pathol 1991 Nov;4(6):733-41. PMID: 1724087

See all (7)

MedGen

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Non-Neoplastic Hematopoietic and Lymphoid Cell Disorder

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Practice guidelines

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