The Out Of Context Mystery

 

 

-A 47 years-old, male

-Known to be diabetic, on oral hypoglycemic

Chronologically:

-18 years ago, he complained of attacks of right hypochondrial pain, radiating to epigastrium and back

-Diagnosed to have calcular gall bladder disease and prescribed medical treatment

-Then he developed jaundice with dark urine, diagnosed to have obstructive jaundice, symptoms relieved with conservative medical treatment and no intervention

-The above scenario repeated typically for three times

-After which a decision was taken to do cholecystectomy

-Pre-operative investigations revealed: severe anemia (Hb 6 g/dl), so surgery was postponed and the patient was referred to a haematologist

-Haemoglobin electrophoresis: Thalassemia trait

-So a decision was taken to have cholecystectomy and splenectomy at the same session after blood transfusion

-Two weeks post-surgery the patient experienced typical chest pain (angina), for which he was admitted to hospital, investigated and stabilized, no interventions were done and discharged on antiplatelets and recommended for outpatient follow-up

-In 2023, he had common cold symptoms for which he was prescribed injection antibiotics, then he had the same previous sequence of symptoms with jaundice: Hb 5 g/dl

-Readmission and investigations: a diagnosis of autoimmune hemolytic anemia

-On presentation to us in 2026: complaining of the same sequence of symptoms (epigastric pain, right hypochondrial pain referring to the back, jaundice) after eating 'heavy meal':

                                                               -T.bilirubin: 4 mg/dl, -ve hepatitis A antibodies test, normal liver enzymes

-After 1 day his jaundice deepened and t.bilirubin reached 39 mg/dl

-So, he presented to us with:

                                    -high-grade fever, rigors

                                    -deep jaundice, t.bilirubin up to 42 mg/dl

                                    -Hb: 6.7 g/dl, T.L.C.:22,000/mm³

-At this point a question raised: does Thalassemia causes this severe picture? So he is out of context thalassemia!

-So, other factors should be sought out

-Other factors like:

                               -other R.B.Cs abnormalities: G6PD, other autoimmune processes,…

                               -Infections, drug intake

-Discussion:

-In view of splenectomy which was done hastily with cholecystectomy

-Splenectomy exposed him to:

                                     -infections

                                     -Thrombocytosis: which typically lasts for about 2 to 3 weeks, reaching a range of 250-500k /mm³ 

-In this patient platelets reached 1.5 million/mm³, lasting for a longer period, causing complications, which is atypical reactive thrombocytosis after splenectomy: out of context thrombocytosis  

-In this picture it caused typical angina which relieved after just antiplatelets

-This marked thrombocytosis also caused: out of context portal vein thrombosis

-Portal vein thrombosis caused cavernous bypassing veins, which may cause compression of the common bile duct

-So, this Cavernoma caused:

                               -Portal Biliopathy  

                               -Portal masses in imaging

-Solution & Resolution:

-So, I decided to intervene with this severe thrombocytosis, as the patient was struggling for a long period with significant complications, even if it is secondary thrombocytosis, besides that antiplatelets treatment is obviously not enough to halt complications.

-So, Hydroxyurea used: promptly platelets dropped to 550k/mm³ and bilirubin to 1.5 mg/dl

Lastly, this is a completely out of context condition:

- Out of context thalassemia trait!

- Out of context thrombocytosis!

- Out of context presentation and complications!

- Out of context in treatment options!