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.
Lymphoid malignancies
| 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 |