Table 1. Reported cases of priapism associated with thalassemia, arranged by chronological order .
WBC, white blood cell; Hb, hemoglobin; HbE, hemoglobin E; NA, not applicable; HU, hydroxyurea; DFX, deferasirox; IV, intravenous; BMT, bone marrow transplant.
| Patient number | Author and year of publication [citation] | Age (years) | Time from onset of priapism to presentation and diagnosis | Type of priapism | Platelet count (x109/L) | WBC (x109/L) | Hb (g/dL) | Type of thalassemia | Intervention/Medication | Splenomegaly/Hepatomegaly | Outcome |
| 1 | Jackson, 1986 [8] | 15 | 10-12h, 4 episodes post-splenectomy | NA | 370 | NA | 8.5 | Intermedia | Hydroxyurea Aspiration | Splenomegaly on initial presentation. | Four recurrent episodes, followed by complete resolution. |
| 2 | Macchia, 1990 [9] | NA | NA | NA | NA | NA | NA | Intermedia | NA | History of splenectomy | NA |
| 3 | Mankan, 2005[10] | 42 | 24 hours | NA | 274 | 6.9 | 10.5 | Thalassemia minor | Aspiration, intracavernous phenylephrine injection | NA | Complete resolution |
| 4 | Sharma, 2008 [11] | 32 | 4 hours prior to current presentation [suffered 20–25 self-remitting similar episodes in last 1 month] | NA | Normal [value not stated]. | Normal [value not stated]. | 10.6 | HbE beta-thalassemia [r IVS1-5[G-C]/b E mutation] | Puncture of corpus cavernosa | Hepatomegaly, history of splenectomy | Complete resolution. |
| 5 | Ziaee, 2008 [12] | 15 | 7 hours | Ischemic | 156.7 | 15.4 | 10 | Major beta-thalassemia | Oxygenation, hydration, epinephrine injections thrice. | History of splenectomy | Complete resolution |
| 6 | Tzortzis, 2009 [13] | 19 | At age of 13 years, then recurrent episodes 6 weeks post-splenectomy | Ischemic stuttering | NA | NA | NA | Thalassemia intermedia | Aspiration, irrigation, self-injections with phenylephrine, oral antiplatelet agent, sildenafil citrate as prevention strategy | Hypersplenism on initial presentation. | Nonpainful recurrent episodes with decreased frequency and not requiring medical management. |
| 7 | Mallat, 2014 [14] | 30 | 1 month | Stuttering priapism | NA | NA | 9.2 | Beta thalassemia intermedia | Cloridrate propanolol 3 drops daily, HU, DFX, cholecalciferol, folic acid | NA | Complete resolution |
| 8 | 35 | 1 month | NA | NA | 7 | Puncture of corpus cavernosa | Hepatomegaly | Complete resolution | |||
| 9 | Öz, 2017 [15] | 17 | Second day of hospitalization | Ischemic, vaso-occlusive | NA | 18.3 | 8.2 | Thalassemia major | IV hydration, alkalization, oxygen therapy, allogeneic BMT, deferosirox as chelation therapy, busulfan, clophosphamide with Mesna | NA | Complete resolution |