Annotation Detail

Information
Associated Genes
ALK
Associated Variants
ALK CLTC-ALK
Associated Disease
diffuse large B-cell lymphoma
Source Database
CIViC Evidence
Description
A 21 year old male presented with lymphadenopathy and a low density mass in pancreas. Histopathological analysis showed diffuse tumor cell infiltrate (CD20/EMA negative) with cytoplasmic ALK. RT-PCR indicated CLTC-ALK fusion. The patient was diagnosed with splenic ALK + diffuse large B cell lymphoma (DLBCL), Stage III, Group A with an IPI of 3. CHOP-21 chemotherapy regimen was administered with improvement in abdominal lymph nodes but worsening in retroperitoneal lymph nodes assessed via standardized uptake values. Subsequent ICE chemotherapy regiment relieved some symptoms prompting the patient to refuse further treatment despite other indications of disease progression. Therapy was reinstated after worsening of disease. GEMOX chemotherapy was administered with dexamethasone and 250mg crizotinib twice daily resulting in symptom relief, decreased lymphadenopathy and LDH decline. The disease then progressed and the patient died 2 months after crizotinib initiation. The authors conclude that partial response to crizotinib was observed and that in future trials ALK rearranged DLBCL patients might see increased benefit with earlier application of targeted therapy during the treatment course.
Variant Origin
somatic
Variant Origin
Somatic
Evidence URL
https://civic.genome.wustl.edu/links/evidence_items/1265
Gene URL
https://civic.genome.wustl.edu/links/genes/1
Variant URL
https://civic.genome.wustl.edu/links/variants/520
Rating
3
Evidence Type
Predictive
Disease
Diffuse Large B-cell Lymphoma
Evidence Direction
Supports
Drug
Crizotinib
Evidence Level
C
Clinical Significance
Sensitivity/Response
Pubmed
26221234
Drugs
Drug NameSensitivitySupported
CrizotinibSensitivitytrue