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Rare Hematological Diseases - Disease Groups

As a result of the revision and discussion of the RHD-DGs during the Board of the Network meeting (13-14 November 2019, Barcelona), a total of 70 RHD-DG has been identified. The Lymphoid malignancies subnetwork includes 19 disease groups encompassing 98 disorders.

RHD-DGs and disorders included are currently under revision for final approval.


Rare bleeding-coagulation disorders and related diseases

Disease Group Disorders included
Acute lymphoblastic leukemia Precursor B-cell acute lymphoblastic leukemia
Precursor T-cell acute lymphoblastic leukemia
   
Diffuse large B-cell lymphoma, Other than NOS Plasmablastic lymphoma
Primary effusion lymphoma
Large B-cell lymphoma with IRF4 rearrangement*
T-cell/histiocyte-rich large B-cell lymphoma
Primary DLBCL of the central nervous system (CNS)
Primary cutaneous DLBCL, leg type
EBV+ mucocutaneous ulcer*
DLBCL associated with chronic inflammation
Lymphomatoid granulomatosis
Primary mediastinal (thymic) large B-cell lymphoma
Intravascular large B-cell lymphoma
ALK+ large B-cell lymphoma
 
Diffuse large B-cell lymphoma, NOS EBV+ DLBCL, NOS*
HHV8+ DLBCL, NOS*
Diffuse large B-cell lymphoma (DLBCL), NOS
Diffuse large B-cell lymphoma (DLBCL), NOS
 
Follicular lymphoma In situ follicular neoplasia*
Duodenal-type follicular lymphoma*
Primary cutaneous follicle center lymphoma
 
Hairy cell leukemia Hairy cell leukemia
 
Histiocytic and dendritic cell neoplasms Histiocytic sarcoma
Langerhans cell histiocytosis
Langerhans cell sarcoma
Indeterminate dendritic cell tumor
Interdigitating dendritic cell sarcoma
Follicular dendritic cell sarcoma
Fibroblastic reticular cell tumor
Disseminated juvenile xanthogranuloma
Erdheim-Chester disease*
 
Hodgkin lymphoma Nodular lymphocyte predominant Hodgkin lymphoma
Classical Hodgkin lymphoma 
Classical Hodgkin lymphoma nodular sclerosis type  
Classical Hodgkin lymphoma mixed cellularity type  
Classical Hodgkin lymphoma lymphocyte-rich type  
Classical Hodgkin lymphoma lymphocyte-depleted type  
 
  Chronic lymphocytic leukemia/small lymphocytic lymphoma
  Monoclonal B-cell lymphocytosis*
  B-cell prolymphocytic leukemia
  Splenic marginal zone lymphoma
Indolent B-cell lymphomas / Non Follicular Splenic B-cell lymphoma/leukemia, unclassifiable
Splenic B-cell lymphoma/leukemia, unclassifiable
  Lymphoplasmacytic lymphoma
  Lymphoplasmacytic lymphoma
  MALT lymphoma
  Nodal marginal zone lymphoma
 
Mantle cell lymphoma Mantle cell lymphoma
 
Mature T-cell neoplasms non primary cutaneous.1 Leukemic Chronic lymphoproliferative disorder of NK cells
T-cell prolymphocytic leukemia
T-cell large granular lymphocytic leukemia
Aggressive NK-cell leukemia
Adult T-cell leukemia/lymphoma
 
Mature T-cell neoplasms non primary cutaneous.2 Extra nodal Enteropathy-associated T-cell lymphoma
Monomorphic epitheliotropic intestinal T-cell lymphoma*
Breast implant-associated anaplastic large-cell lymphoma*
Extranodal NK-/T-cell lymphoma, nasal type
Indolent T-cell lymphoproliferative disorder of the GI tract*
Hepatosplenic T-cell lymphoma
 
Mature T-cell neoplasms non primary cutaneous.2 Nodal Peripheral T-cell lymphoma, NOS
Angioimmunoblastic T-cell lymphoma
Anaplastic large-cell lymphoma, ALK+
Anaplastic large-cell lymphoma, ALK−*
Nodal peripheral T-cell lymphoma with TFH phenotype*
Follicular T-cell lymphoma*
 
  Subcutaneous panniculitis-like T-cell lymphoma
  Mycosis fungoides
  Sézary syndrome
Primary cutaneous CD30+ T-cell lymphoproliferative disorders
Mature T-cell neoplasms primary cutaneous Primary cutaneous CD30+ T-cell lymphoproliferative disorders
  Primary cutaneous γδ T-cell lymphoma
  Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma
  Primary cutaneous acral CD8+ T-cell lymphoma*
  Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder*
 
Other agressive B-cell neoplasms Burkitt lymphoma
Burkitt-like lymphoma with 11q aberration*
High-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements*
High-grade B-cell lymphoma, NOS*
B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and classical Hodgkin lymphoma
 
  Monoclonal gammopathy of undetermined significance (MGUS), IgM*
  μ heavy-chain disease
γ heavy-chain disease
  α heavy-chain disease
  Monoclonal gammopathy of undetermined significance (MGUS), IgG/A*
Plasma cell neoplasm Solitary plasmacytoma of bone
  Extraosseous plasmacytoma
  Monoclonal immunoglobulin deposition diseases*
  POEMS syndrome
  Plasma cell leukemia
 
Plasma cell myeloma (not rare) Plasma cell myeloma
AL amyloidosis AL amyloidosis
 
Posttransplant lymphoproliferative disorders (PTLD) Plasmacytic hyperplasia PTLD
Infectious mononucleosis PTLD
Florid follicular hyperplasia PTLD*
Polymorphic PTLD
Monomorphic PTLD (B- and T-/NK-cell types)
Classical Hodgkin lymphoma PTLD

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