AHMED KADHIM THALASSEMIA CENTER. TOPICS CASE PRESENTATION Hb SD DISEASE OSTEONECROSIS (avasculer necrosis of bone) THROMBOCYTOSIS IN Hb S PATIENT.

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Presentation transcript:

AHMED KADHIM THALASSEMIA CENTER

TOPICS CASE PRESENTATION Hb SD DISEASE OSTEONECROSIS (avasculer necrosis of bone) THROMBOCYTOSIS IN Hb S PATIENT

CASE PRESENTATION - HISTORY Male, UAE national Age 15 yrs, (D.O.B- Aug. 1993) Age 4 ms. - anemia, jaundice, diagnosis made (Hb electrophoresis and family studies), first blood transfusion. Age 1.5 yrs. (Nov. 1994) - 1 st seen in our center. Age 2.5 yrs. (Nov. 1995) – kept on regular blood transfusion and iron chelation (Desferal).

CASE PRESENTATION - HISTORY Age 7.5 yrs (Nov. 2000) – splenectomy (??BMT). Age 8.5 yrs (Dec. 2001) – iron chelation stopped (ferrtin 300), on demand blood transfusion. Age 9-11 yrs (Apr Jul. 2004) – ACS, 4x. Age 12 yrs. (Jul. 2005) – cholecystectomy.

CASE PRESENTATION - HISTORY Age 13 yrs (Jul. 2007) – hip pain, limping, restricted movement. - X ray, MRI, orthopedics opinion. - Hydroxyurea 15mg/kg/d, zolindronic acid, blood Tx.

Age 14 yrs.(Nov. 2007) – osteotomy, Rt. Hip joint. - pain free, no more limping.

CASE PRESENTATION - HISTORY Age yrs (Mar May 2009) – hospitatalized for pain episodes, 4-6x.

CASE PRESENTATION - CLINICAL. May Weight 3 rd, height 10 th. - Early pubertal - Mild jaundice, scars. - Hip joint - painless, full range movement. Management – Hydroxyurea 20 mg/kg/d, exchange Tx.(partial).

CASE PRESENTATION – LAB. TESTS. HU

CASE PRESENTATION – LAB. TESTS. HU

CASE PRESENTATION – LAB. TESTS. HU

CASE PRESENTATION – LAB. TESTS. HU

CASE PRESENTATION – LAB. TESTS. HU

CASE PRESENTATION – LAB. TESTS. HU

CASE PRESENTATION – LAB. TESTS. HU

CASE PRESENTATION – LAB. TESTS. HU

CASE PRESENTATION – LAB. TESTS. HU

PLAN HYDROXYUREA, max. tolerated dose ? Exchange transfusion ? Bone marrow transplantation ?

HB D 1939 – USA, 1 st family. β 121 lys. Glutamate. CD 121 (GAG GTG) USA – D Los Angeles, UK – D Punjab Afro-American, Afro-Caribbean, Central and South America, North Eurepean, English,Turks, Mediterranean, Indian. Heterozygous – asymptomatic. Homozygous – rare, mild hemolysis. Hb D Iran, Ibadan, asymptomatic variants.

HB D – SIGNIFICANCE 1 Electrophoresis (alkaline) – like Hb S, Hb G philadilphia Hb SS = Hb DD = Hb SD Sickling test. Hb solubility test. Electrophoresis (acid). HPLC.

HB D – SIGNIFICANCE 2 Hb SD disease – milder than sever sickle cell anemia. - splenemegaly. - Hb 5-10 g/dl. - MCV variable, macrocytosis common. - Diagnosis HPLC. - Hb D > Hb S. - ONLY Hb D Punjab/Los Angeles variant. - Haemoglobinopathy diagnosis, Barbara J. Bain 2003.

Osteonecrosis - overview cellular death of bone components due to interruption of the blood supply; the bone structures then collapse, resulting in bone destruction, pain, and loss of joint function. USA, NEW CASES YEARLY. Africo american. M:F 8:1. Symptoms and signs – not at the early stage.

Osteonecrosis - CAUSES Causes - Trauma,Systemic corticosteroid use or Cushing disease,Alcohol abuse,Systemic lupus erythematosus Hematologic (sickle cell disease, hemoglobinopathies) Metabolic (hyperlipidemia, gout, renal failure) Orthopedic disorders,Infection,Renal transplantsplant Radiation therapy,Pancreatitis,Gaucher disease Malignancy,Caisson disease,Pregnancy,Bisphosphonate use

Osteonecrosis - STAGING Stages – 0- VI. Symptoms – II. X ray – II. MRI – I. Surgery unlikely to preserve the joint beyond stage III.

Osteonecrosis IMAGING.

Osteonecrosis - Management. Medical (early)– pain control, immobilization, underlying cause, bisphosphonate, statins(prevention in CS therapy). Surgical - core decompression, bone graft, osteotomy, total hip replacement.

THROMBOCYTOSIS Acute phase reaction? Common in sicklers? Splenectomy? Underlying pathology, MPD?

THANK YOU