Critical Care and Shock

An uncommon hematologic disorder in an adult Puerto Rican patient

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We described a 24 years old man, born in Puerto Rico, with a history of type two diabetes mellitus, hypothyroidism and morbid obesity admitted after complaining of general malaise, dizziness, confusion, and unquantified fever of two weeks evolution. Pertinent clinical findings included hepatosplenomegaly and hypotension. Laboratories demonstrated leukocytosis, normocytic anemia, thrombocytopenia, high ferritin, increased lactate dehydrogenase (LDH) and elevated creatinine level along with hypertriglyceridemia. Further laboratories revealed bacteremia with Methicillin Resistant Staphylococcus aureus (MRSA). An abdominal sonogram confirmed an enlarged liver measuring 23.6 cm and a spleen 18.2 cm long. A bone marrow biopsy evidenced histiocytes with engulfed red cells and nucleated cells.

The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) secondary to bacteremia with MRSA. He coursed with severe sepsis involving target organs such as kidneys, bone marrow, liver and spleen. Daptomycin and Methylprednisolone were prescribed for the bacteremia and the hematologic condition respectively, with a favorable overall clinical response.

HLH is an uncommon disorder with an estimated incidence of 1 in 50,000 lives births, having an equal male to female distribution. The current incidence in adults is unknown. Therefore, we like to share this case of an immune-competent, Hispanic adult with no history of familial immune deficiencies condition who presented an HLH secondary to bacteremia.

To our knowledge, there are no published studies describing this syndrome in an immune-competent adult from Puerto Rico, making this case very uncommon in our island.

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