Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA
© 2017 The Korean Society of Pathologists/The Korean Society for Cytopathology
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Conflicts of Interest
No potential conflict of interest relevant to this article was reported.
IVLBCL, intravascular large B-cell lymphoma; M, male; GCB, germinal center B cell; F, female; IVIG, intravenous immunoglobulin; R-CHOP, rituximab-cytoxan, doxorubicin, vincristine and prednisone; R-MPV, rituximab-methotrexate, procarbazine and vincristine; aSCT, autologous stem cell transplant; CNS, central nervous system; N/A, not available.
Case | Age (yr)/Sex | Clinical presentation | Sites of involvement | Cell of origin | Bone marrow involvement | Treatment and outcome |
---|---|---|---|---|---|---|
1 | 81/M | Respiratory failure | Lungs, heart, adrenal glands, kidneys, prostate, bladder esophagus, stomach, pancreas, intestines | Non-GCB | No | None; deceased prior to diagnosis. |
2 | 76/F | Encephalopathy | Brain, skin, breast, heart, lungs, liver, adrenal glands, stomach, pancreas, intestines | Non-GCB | Yes | None; deceased prior to diagnosis |
3 | 84/M | Fever and chills | Adrenal glands, bladder, stomach, pancreas, intestines, appendix, liver | Non-GCB | No | IVIG, steroids, deceased shortly after diagnosis |
4 | 61/F | Progressive skin rash; relapse 6 mo later presenting as loss of consciousness | Skin, brain | Initial: non-GCB | No | Status post R-CHOP, R-MPV, aSCT; disease-free at last follow-up (22 mo after CNS recurrence) |
Relapse: non-GCB | ||||||
5 | 56/F | Skin rash | Skin | Non-GCB | No | N/A (outside consultation) |
Immunoglobulin gene rearrangement by PCR |
|||
---|---|---|---|
IgH | IgK (tube A) | IgK (tube B) | |
Case 1 | Negative | Positive (272 bp) | Positive (239 bp) |
Case 2 | Positive (319 bp, 254 bp) | Positive (149 bp, 201 bp) | Positive (275 bp) |
CNS: meninges | Negative | Positive (149 bp, 201 bp) | Positive (275 bp) |
CNS: pituitary | Positive (254 bp) | Negative | Negative |
Case 3 | Negative | Negative | Negative |
Case 4 | Negative | Negative | Negative |
Case 5 | Negative | Positive (149 bp) | Positive (237 bp) |
IVLBCL, intravascular large B-cell lymphoma; M, male; GCB, germinal center B cell; F, female; IVIG, intravenous immunoglobulin; R-CHOP, rituximab-cytoxan, doxorubicin, vincristine and prednisone; R-MPV, rituximab-methotrexate, procarbazine and vincristine; aSCT, autologous stem cell transplant; CNS, central nervous system; N/A, not available.
IVLBCL, intravascular large B-cell lymphoma; PCR, polymerase chain reaction; CNS, central nervous system.