Table 3.
Hematologic and Lymphatic Features
| PI3KCD n=33 | PI3KR1 n=7 | Combined n=40 | |
|---|---|---|---|
| NON-MALIGNANT | |||
|
| |||
| Splenomegaly | 18 (54.5%) | 4 (57.1%) | 22 (55.0%) |
|
| |||
| Lymphadenopathy | 16 (48.5%) | 5 (71.4%) | 21 (52.5%) |
|
| |||
| Hepatomegaly | 9 (27.2%) | 2 (28.6%) | 11 (27.5%) |
|
| |||
| Hemolytic Anemia | 7 (21.2%) | 0 | 7 (17.5%) |
|
| |||
| Nodular Lymphoid Hyperplasia | 4 (12.1%) | 0 | 4 (10.0%) |
|
| |||
| Pancytopenia | 4 (12.1%) | 0 | 4 (10.0%) |
|
| |||
| Immune Thrombocytopenia | 3 (9.1%) | 1 (14.3%) | 4 (10.0%) |
|
| |||
| Factor XI deficiency | 2 (6.1%) | 0 | 2 (5.0%) |
| Factor IX deficiency | 1 (3.0%) | 0 | 1 (2.5%) |
|
| |||
| Spherocytosis | 1 (3.0%) | 0 | 1 (2.5%) |
|
| |||
| TTP | 1 (3.0%) | 0 | 1 (2.5%) |
|
| |||
| MALIGNANT | |||
|
| |||
| Lymphoma – All | 4 (12.1%) | 1 (14.3%) | 5 (12.5%) |
|
| |||
| Non-Hodgkin’s Lymphoma | 1 (3.0%) | 1 (14.3%) | 2 (5.0%) |
|
| |||
| Hodgkin’s Lymphoma | 1 (3.0%) | 0 | 1 (2.5%) |
|
| |||
| Diffuse large B cell lymphoma | 2 (6.1%) | 0 | 2 (5.0%) |
|
| |||
| MALT lymphoma | 2 (6.1%) | 0 | 2 (5.0%) |
|
| |||
| Other | - Hurthle cell carcinoma of thyroid (1) - Dysgerminoma (1) |
- Dysgerminoma (1) | 3 (7.5%) |
Benign lymphoid hyperplasia was commonly reported. Five patients (12.5%) developed lymphoma. The history of lymphoma was significantly associated with EBV (P=0.004) but was not with other herpesviruses (P=0.056).