Uncommon presentations of MCL that may be underrepresented in prospective interventional clinical trials
| Type of MCL . | Presentation . |
|---|---|
| Indolent | May present with leukemic, nonnodal MCL and wild-type TP53 or low-tumor-burden–typical MCL with low Ki-67 expression, simple karyotype, normal LDH, and lack of B symptoms. There is no clear disadvantage to deferred therapy. |
| Leukemic, nonnodal | Presents with minimal adenopathy but splenomegaly and lymphocytosis, which is frequently IGHV mutated/SOX11-negative. Mutated TP53 predicts poor prognosis. |
| Super-high risk | Key predictive factors include an elevated MIPI score and mutated TP53. Other features typically include blastoid histology and high Ki-67 expression. Prognosis is poor despite intensive therapy. |
| High risk for CNS involvement | Blastoid histology and high Ki-67 expression are the most commonly reported risk factors. The role of prophylaxis is unclear. |
| Type of MCL . | Presentation . |
|---|---|
| Indolent | May present with leukemic, nonnodal MCL and wild-type TP53 or low-tumor-burden–typical MCL with low Ki-67 expression, simple karyotype, normal LDH, and lack of B symptoms. There is no clear disadvantage to deferred therapy. |
| Leukemic, nonnodal | Presents with minimal adenopathy but splenomegaly and lymphocytosis, which is frequently IGHV mutated/SOX11-negative. Mutated TP53 predicts poor prognosis. |
| Super-high risk | Key predictive factors include an elevated MIPI score and mutated TP53. Other features typically include blastoid histology and high Ki-67 expression. Prognosis is poor despite intensive therapy. |
| High risk for CNS involvement | Blastoid histology and high Ki-67 expression are the most commonly reported risk factors. The role of prophylaxis is unclear. |
LDH, lactate dehydrogenase.