Haematologica 2002; 87:(11)ECR36
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Hypopituitarism in a patient with intravascular lymphomatosis
Price DA, Thaker H, James A, Snow MH
Dept. of Infectious Diseases and Tropical medicine, Newcastle General Hospital, West Rd, Newcastle-Upon-Tyne, UK. E-mail: ashley.price@nuth.northy.nhs.uk

We read with interest the report by Schleinitz N et al. Two cases of intravascular lymphomatosis revealed by hypopituitarism1 and would like to present a similar case which illustrates the systemic nature of intravascular lymphomatosis, difficulties in the diagnosis and that the condition may lead to hypopituitarism.
Case Report. The patient, a 57-year old woman, presented to outpatients complaining of nausea and poor appetite associated with one kilogram of weight loss over 4 months. She felt depressed and lacked energy. She also had episodic sweats and fevers.She looked pale and unwell and had periorbital and sacral edema. She was hypotensive (BP 94/60) and tachycardic (PR 100/min). An underlying neoplasia was suspected. She was found to have spiking temperatures of 40°C. Investigations revealed a normochromic normocytic anemia (Hb10.9 g/dL), thrombocytopaenia (platelets 84) and lymphopaenia (0.4). Her inflammatory markers were raised (CRP 70.1, ferritin 4473, ESR 40). She had raised urate and lactate dehydrogenase (4400 u/L) and b2-microglobulin (7.7 mg/L (normal 1.0-3.0 mg/L)) levels. Her CXR, electrolytes, liver function tests and serum electropheresis were normal. Because of the pyrexia and hypotension she was started on intravenous teicoplanin and ceftazidime for possible sepsis. There was no response in her condition. Her thyroid function tests revealed a low thyroid stimulating hormone level (TSH) and free T4 level. Due to her hypotension and loss of weight she had a tetracosactrin test, which showed a poor adrenal response. She was started on hydrocortisone 50 mg tds. Further pituitary function tests were performed and revealed anterior pituitary failure (Table 1).
An ultrasound scan of her abdomen revealed an enlarged spleen. A trephine bone marrow biopsy revealed hypercellularity and normal at hemopaerisis with areas of necrosis and gelatinous degeneration.
She deteriorated with worsening hypotension, tachycardia, pyrexia and edema and developed a pleural effusion and ascites. A white cell scan revealed increased uptake in the spleen, left iliac fossa and both lungs.
An echocardiogram showed moderately severe impairment of her left ventricle with a small pericardial effusion. Her IV hydrocortisone was increased to 50 mg qds and she went on to have a CT scan of her chest and abdomen, which did not reveal any further abnormalities.
She was transferred to ITU. A right heart catheter was placed; she had a wedge pressure of 12 and a cardiac output of 4.7. Fluid resuscitation did not improve her hypotension and she developed broad complex tachyarryhthmias. Her hypotension was refractory to high dose dopamine, dobutamine and noradrenaline and she died. A post-mortem revealed a systemic angiocentric large B cell lymphoma with multi-organ infiltration, including the pituitary, lungs and heart.
Discussion. Intravascular lymphomatosis with intracranial involvement is a rare condition. Intravascular lymphomatosis most commonly affects the central nervous system. It may affect the skin, liver, lung, adrenals, spleen, heart and bone marrow. As in our patient diagnosis is often only made at post-mortem. When diagnosed antemortem treatment has been attempted successfully with chemotherapy, the most commonly used regimen has been CHOP.1-3 High dose chemotherapy and autologous peripheral stem cell transplantation has been a successful treatment Ref4,5.
Hypopituitarism has been described previously in four patients with intravascular lymphomatosis.1,2,6 Our patient presented with pyrexia of unknown origin and was found to have hypopituitarism. She went on to develop multiorgan failure. There was cardiovascular dysfunction with shock unresponsive to ionotropes and a broad complex tachycardia due to cardiac infiltration and respiratory failure due to pulmonary invasion.

References

  1. Schleinitz N, Bernit E, Mazodier K. Two cases of intravascular lymphomatosis revealed by hypopituitarism. Haematologica 2002; 87:(06)ECR21.
  2. Taura Y, Yamazaki H, Katou T. Two cases of intravascular lymphomatosis diagnosed antemortem by transbronchial biopsy. Nihon Kokyuki Gakkai Zasshi 2000; 38: 34-8.
  3. Baumann TP, Hurwitz N, Karamitopolou-Diamantis E. Diagnosis and treatment of intravascular lymphomatosis. Archives Neurology 2000; 57: 374-7.
  4. Dubas F, Saint-Andre JP, Pouplard-Barthelaix A, et al. Intravascular malignant lymphomatosis (so-called malignant angioendol theliomatosis): a case confined to the lumbosacral spinal cord and nerve roots. Clin Neurophysiol 1990:115-20.
  5. Smadja D, Mas JL, et al. Intravascular lymphomatosis (neoplastic angioendotheliosis) of the central nervous system: case report and literature review. J Neurooncol 1991; 11: 171-80.
  6. Yamaguchi M, Kimura M et al. Successful autologous peripheral blood stem cell transplantation for relapsed intravascular lymphomatosis. Bone Marrow Transplant 2001; 27: 89-91.
  7. Koizumi M, Nishimura M et al. Successful treatment of intravascular malignant lymphomatosis with high-dose chemotherapy and autologous peripheral blood stem cell transplantation. Bone Marrow Transplant 2001; 27: 1101-3.