Details of Disease

General Information of Disease (ID: DISDPPHX)

• Disease Name: Prolymphocytic leukaemia
• Synonyms: prolymphocytic leukemia (B or T-cell); prolymphocytic leukemia (B or T); prolymphocytic leukemia; prolymphocytic leukaemia (B or T-cell); prolymphocytic leukaemia (B or T); PLL
• Disease Class: 2A82: Mature B-cell leukaemia
• Definition: A mature B- or T- cell leukemia with progressive clinical course. It is characterized by the presence of medium-sized lymphocytes with visible nucleoli (prolymphocytes) in the peripheral blood, bone marrow, and spleen.
• Disease Hierarchy:
  DIS0C4XI: Chronic leukemia
  DIS87TGM: Acute lymphoblastic leukaemia
  DISDPPHX: Prolymphocytic leukaemia
• ICD Code:
  ICD-11: ICD-11: 2A82.1
  ICD-10: ICD-10: C91.3
  Expand ICD-11: 'XH8TD6
• Disease Identifiers:
  MONDO ID: MONDO_0001023
  MESH ID: D015463
  UMLS CUI: C0023486
  MedGen ID: 6062
  SNOMED CT ID: 110006004

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 4 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Lenalidomide	DM6Q7U4	Approved	Small molecular drug	[1]
Rituximab	DM1YVZT	Approved	Antibody	[2]
Sunitinib	DMCBJSR	Approved	Small molecular drug	[3]
Thalidomide	DM70BU5	Approved	Small molecular drug	[4]

This Disease is Treated as An Indication in 6 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
APG-115	DMIFA0L	Phase 2	NA	[5]
CART-19	DMHQKN6	Phase 2	CAR T Cell Therapy	[6]
Anti-CD19-CAR vector-transduced T cells	DMQXU4C	Phase 1/2	CAR T Cell Therapy	[7]
Anti-CD19/22-CAR vector-transduced T cells	DMMUSHR	Phase 1/2	CAR T Cell Therapy	[8]
Anti-CD20-CAR vector-transduced autologous T cells	DMCDUJY	Phase 1/2	CAR T Cell Therapy	[9]
Anti-CD19/20-CAR vector-transduced T cells	DM3M9OY	Clinical trial	CAR T Cell Therapy	[10]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 2 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
TCL1A	TTUKRDV	Limited	Genetic Variation	[11]
DUSP5	TTZN92A	Strong	Altered Expression	[12]

This Disease Is Related to 3 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
TCL1B	OT4CSO39	Limited	Genetic Variation	[11]
KLRC2	OTT4N86S	Strong	Biomarker	[13]
MARCKSL1	OT13J2FM	Strong	Altered Expression	[14]

References

• [1] Lenalidomide FDA Label
• [2] B and T cell prolymphocytic leukaemia. Best Pract Res Clin Haematol. 2019 Sep;32(3):217-228.
• [3] Sunitinib FDA Label
• [4] Thalidomide FDA Label
• [5] ClinicalTrials.gov (NCT04496349) A Study Evaluating APG-115 as a Single Agent or in Combination With APG-2575 in Subjects With T-PLL. U.S. National Institutes of Health.
• [6] ClinicalTrials.gov (NCT01029366) CART19 to Treat B-Cell Leukemia or Lymphoma That Are Resistant or Refractory to Chemotherapy
• [7] ClinicalTrials.gov (NCT01864889) Treatment of Relapsed and/or Chemotherapy Refractory B-cell Malignancy by CART19
• [8] ClinicalTrials.gov (NCT03185494) Treatment of Relapsed and/or Chemotherapy Refractory B-cell Malignancy by Tandem CAR T Cells Targeting CD19 and CD22
• [9] ClinicalTrials.gov (NCT01735604) Genetically Engineered Lymphocyte Therapy in Treating Patients With Lymphoma That is Resistant or Refractory to Chemotherapy
• [10] ClinicalTrials.gov (NCT03097770) Treatment of Relapsed and/or Chemotherapy Refractory B-cell Malignancy by Tandem CAR T Cells Targeting CD19 and CD20
• [11] Incidence of TCR and TCL1 gene translocations and isochromosome 7q in peripheral T-cell lymphomas using fluorescence in situ hybridization.Am J Clin Pathol. 2008 Aug;130(2):178-85. doi: 10.1309/PNXUKA1CFJMVGCN1.
• [12] High frequency of somatic mutations in the VH genes expressed in prolymphocytic leukemia.Blood. 1996 Nov 15;88(10):3953-61.
• [13] Pretransplant adaptive NKG2C+ NK cells protect against cytomegalovirus infection in kidney transplant recipients.Am J Transplant. 2020 Mar;20(3):663-676. doi: 10.1111/ajt.15658. Epub 2019 Nov 19.
• [14] Multidrug resistance-associated protein (MRP) in haematological malignancies.Leuk Lymphoma. 1996 Feb;20(5-6):381-7. doi: 10.3109/10428199609052419.