Track 19

World Hematology 2018, track 19 is all about Hematology/ oncology Case reports.

Unleash the enigma in Hematology/ Oncology through case reports. Here we go for an example case report.

Sickle Cell Beta-Plus Thalassemia with Subcapsular Hematoma of the Spleen

Introduction:

Sickle-cell disease (SCD) is a group of blood disorders typically inherited from a person’s parents. The most common type is known as sickle-cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin (haemoglobin S) found in red blood cells.

It affects millions of people worldwide and is particularly most prevalent in people whose ancestors came from sub-Saharan Africa, Central America, South America, Saudi Arabia, India, and Mediterranean countries. 

Case report:

A 32-year-old male patient went to a hospital complaining of dull left upper quadrant pain of 2 days duration. He denied any recent trauma. He had no fever, chills, sore throat, malaise, arthralgia, skin rash, or bleeding from the gums or any other site. The rest of the review of symptoms was negative. His past medical history was significant for an unclear sickling disorder. The patient himself reported having sickle cell trait and getting exchange transfusions as a child. His family history was significant for SCD in mother.

His vitals were stable at presentation. Physical examination was significant for splenomegaly and mild tenderness at the left upper quadrant of the abdomen with no icterus, hepatomegaly, or lymphadenopathy. Laboratory tests showed a leukocyte count of 10,400/L (normal 4,500–11,000/L), hemoglobin of 13.4 gm/dL (normal 13.5–17.5 gm/dL), hematocrit of 41% (normal 41%–53%) with mean corpuscular volume of 66.8 fL (normal 80–100 fL), platelet count of 78,000/L (normal 130,000–400,000/L), activated partial thromboplastin time of 35.7 seconds (normal 24.9–35.9 seconds), serum blood urea nitrogen of 13 mg/dL (normal 8–20 mg/dL), serum creatinine of 1 mg/dL (normal 0.4–1.3 mg/dL), total bilirubin of 2.2 mg/dL (normal 0.3–1.2 mg/dL), aspartate aminotransferase of 28 IU/L (normal 15–41 IU/L), alanine aminotransferase of 33 IU/L (normal 17–63 IU/L), and serum alkaline phosphatase of 56 IU/L (normal 32–91 IU/L). Further workup for microcytic anemia showed a reticulocyte count of 1.96% (normal 0.5%–2%), serum haptoglobin of 62 mg/dL (normal 34–200 mg/dL), serum lactate dehydrogenase of 245 IU/L (normal 98–192 IU/L), serum iron of 33 ug/dL (normal 38–169 ug/dL), iron saturation of 16% (normal 15%–55%), serum ferritin of 269 ng/mL (normal 30–400 ng/mL), and a positive sickle cell screen. Subsequent hemoglobin electrophoresis revealed 17.5% of hemoglobin A (normal 94%–98%), 7.2% of hemoglobin A2 (normal 0.7%–3.1%), 73.8% of hemoglobin S (normal 0%), and 1.5% of hemoglobin F (normal 0–2%), which was suggestive of sickle cell beta-plus thalassemia. Computed tomography (CT) scans of the abdomen showed splenomegaly with multiple splenic subcapsular lacerations and hematomas. No other possible cause for spontaneous splenic rupture was found in the patient. His symptomatology, physical examination, and initial lab tests were not consistent with the common causes of splenic rupture like hematological malignancies, hemophilia, or malaria. Tests for Epstein–Barr virus, cytomegalovirus, human immunodeficiency virus, and hepatitis A, B, and C viruses returned negative. So, with the diagnosis of spontaneous splenic rupture with multiple subcapsular hematomas in a patient with sickle cell beta-plus thalassemia, the patient was initially managed conservatively with bed rest, analgesia, multivitamin, folic acid, and pneumococcal and meningococcal vaccinations with plans for splenectomy going forward.

However, on the fourth day of admission, the patient started to have cough, productive of yellowish sputum and palpitation. He had low-grade fever, tachycardia, and tachypnea. Repeat CT scan of the chest showed new bilateral lower lobe infiltrates. Arterial blood gas analyses in room air showed a pH of 7.46 (normal 7.35–7.45), pCO₂ of 37 mmHg (normal 35–45 mmHg), pO₂ of 100 mmHg (normal 75–100 mmHg), and HCO₃ of 26.2 mmol/L (normal 18–24 mmol/L). So, with the possible diagnosis of acute chest syndrome and/or hospital-acquired pneumonia, the patient underwent exchange transfusion with 2.8 liters of leukocyte-reduced red blood cell and broad-spectrum antibiotics. Subsequently his symptoms resolved, and his vital signs stabilized. The patient, however, refused splenectomy during his hospital stay. And with his hematological parameters stable, he was discharged home with a leukocyte count of 7,400/L, hemoglobin of 10.7 gm/dL, hematocrit of 32.5%, and platelets count of 263,000/L at discharge.

Conclusion:

Splenic rupture and hematoma mimicking acute abdomen may rarely be the only presenting feature in patients with sickle cell disease and its variants like sickle cell beta⁺ thalassemia as seen in our case. Clinicians, therefore, need to be aware of this rare complication and consider hemoglobin electrophoresis a part of the workup for unexplained cases of nontraumatic splenic hematoma.

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