Case 32 Backing the wrong horse

Mr Moses, a 46-year-old primary school teacher of Afro-Caribbean origin, was referred to the medical take with right-sided ear ache, headache and general malaise on a background of a three week history of right facial weakness. The latter had been treated by Mr Moses’s GP as probable Bell’s palsy with steroids and aciclovir.

Mr Moses had a history of genital herpes two years prior. Travel history included a camping trip to the New Forest three months ago.

On examination, Mr Moses was found by the SHO to be drowsy but easily roused. His facial muscles appeared generally weak (lower motor neurone pattern) and he was unsteady on his feet. There was some neck stiffness and plantars were down-going.

What would you advise?

In view of the bilateral facial palsy and the deteriorating conscious level, the Consultant considered meningo-encephalitis secondary to either herpes infection or Lyme disease to be the likely diagnosis. Broad spectrum antibiotics and aciclovir were given pending CT and a lumbar puncture.

Prior to lumbar puncture, serum calcium was found to be elevated at 3.03 and a chest X-ray demonstrated the presence of bilateral hilar enlargement. The working diagnosis was revised to neurosarcoidosis. Prednisolone was recommenced and fluids were administered.

The next day, a subtle papular rash began to develop. Skin and conjunctival biopsies were arranged, along with serum ACE levels and an MRI scan of the parotids, in order to assist in the confirmation of a diagnosis of sarcoidosis. The patient was discharged to return to the outpatient clinic for review and results five days later.

Any other possible diagnoses?

Mr Moses returned to hospital the day before his planned clinic appointment with insomnia, leg weakness and a metallic taste in the mouth. A number of small lymph nodes were identified on examination and a biopsy was arranged. Results from the previous admission were now available including serum ACE within the normal range and a skin biopsy consistent with fungal infection. Repeat bloods showed serum calcium of 3.56 and a white cell count elevated at 25 000. A blood film demonstrated the presence of circulating lymphoblasts.

A clinical haematology opinion was organized and a working diagnosis of HTLV-1 associated adult T-cell lymphoblastic lymphoma was made.

Mr Moses received urgent systemic chemotherapy with intra-thecal methotrexate. He subsequently relapsed and received several more courses of chemotherapy. No HLA-matched donor was available and Mr Moses died eight months after diagnosis.

Expert opinion

It is possible that Mr Moses’s diagnosis could have been made a number of days earlier had the link between hypercalcaemia in a patient of afro-Caribbean origin and T-cell lymphoblastic lymphoma been appreciated by the admitting team. This link is readily recognized within haematological circles but not amongst general physicians.

The admitting team, and the other specialties called upon to provide an opinion, were all rather blinkered in their quest to ‘confirm sarcoid’ rather than to approach the case from a more objective standpoint and constructively question that working diagnosis at every turn. It seems that the lymphadenopathy identified on readmission had developed very acutely along with the peripheral blood abnormalities. These two findings made the possibility of haematological malignancy obvious and it is difficult to avoid use of a ‘retrospectoscope’ once it is acquired. It is unclear whether a subtler degree of lymphadenopathy was present and should have been detected upon the initial presentation.

In Mr Moses’s case, the short delay in the diagnosis of a rare but devastating illness was of no consequence – appropriate measures had been put in place to manage the hypercalcaemia and appropriate follow up had been arranged to see through the result of the investigations that had been ordered.

Legal comment

When does making a clinical error equate to negligence? The recognition of the link between hypercalcaemia and T-cell lymphoma is recognized in the clinical subspecialty of haematology. When assessing the duty of care of the general physician, for example, it is unlikely that a responsible body of medical opinion would have been aware of the link. As such, this failure to diagnose is unlikely to have been negligent. In this case even though there was an error in diagnosis, the causative link, between this admission and patient’s death does not exist. On the balance of probability, the outcome would have been the same in any event. Any error was not the single direct cause of the patient’s death.

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