Lathyrism, Leg Cramps, and Thrombocytopenia: Cascade of Events Starting in a Concentration Camp
Article Outline
To the Editor:
An accurate history of patient illness remains the main approach for a correct diagnosis. We report the peculiar evolution of thrombocytopenic purpura in an elderly woman and the relationship with a forgotten illness, lathyrism.
An 85-year-old Jewish woman born in Romania presented in 1998 with purpura and epistaxis caused by severe thrombocytopenia (2
×109/L). She had hypertension and hypothyroidism that were treated with enalapril and thyroxine. Laboratory tests revealed a hemoglobin level of 12.2 g/dL and white blood cell count of 9.2×109/L. Serology for infectious and collagen diseases was negative. Bone marrow aspiration showed normal hematopoiesis and increased megakaryocyte number, compatible with peripheral destruction of platelets. The diagnosis of immune thrombocytopenia was made, but its etiology remained obscure. The patient received a single platelet transfusion for bleeding management, and 1 week later her platelet count was 240×109/L. Three months later, she had a new episode of bleeding with thrombocytopenia (4×109/L) and again recovered after a platelet transfusion.
The puzzling course of abrupt, severe, and short episodes of thrombocytopenia urged us to further inquire about the patient’s history and medications. She remembered occasionally taking quinine for leg cramps. She also added that 50 years ago, during detention in a concentration camp, she was diagnosed with a mild form of lathyrism. The relationship among lathyrism, leg cramps, quinine, and acute thrombocytopenia was established. Moreover, the thromboagglutinin test result with quinine was positive.
Lathyrism is a neurologic disorder caused by excessive or long-term consumption of the grass pea Lathyrus sativus.1 Since September 1942, bread made from the flour of this plant was given to prisoners in Vapniarca, a German concentration camp for Romanian Jews. After 3 months, lathyrism developed in 800 people.2
Lathyrism was first reported 2000 years ago by the Indian physician Charak, followed by Hippocrates, who associated eating L. sativus with the occurrence of neurologic disturbances. Currently, the disease is forgotten in western countries and limited to endemic areas such as India and Ethiopia. The pea can be boiled and eaten as gruel or ground into flour to make bread. The seeds contain protein, but unfortunately they also contain a number of toxic compounds. One of them, beta-N-oxalyl-diaminopropionic acid, is a strong stimulator of glutamate receptors in the nervous system.1 Neurolathyrism is characterized by a motor hypertonic paresis, in which the legs are affected more than the arms. Early symptoms include walking difficulties, muscle cramps, and leg weakness. Later it produces irreversible spastic paralysis and even death due to failure of the respiratory system muscles. Vascular damage (angiolathyrism) and bone growth damage in children (osteolathyrism) have also been described. There is no cure for lathyrism.
Our patient occasionally used quinine for relief of leg cramps. Stopping drug administration ceased the occurrence of thrombocytopenia for the following 8 years. Quinine was implicated in the earliest reported causes of drug-induced thrombocytopenia.3 Usually the thrombocytopenia occurs rapidly after the initial drug administration, although it may occur months or years later.4
This case emphasizes the importance of a complete medical history. Any event related by the patient may be the key to solving his or her health problem.
References
- Lathyrism: evidence for role of the neuroexcitatory amino acid BOAA. Lancet. 1986;8515:1066–1067
- . Lathyrismus. Monatsschr Psychiatri Neurol. 1947;113:345–376
- . Quinine as a cause of purpura. Lancet. 1865;2:37
- . Spontaneous reports of thrombocytopenia in association with quinine: clinical attributes and timing related to regulatory action. Am J Hematol. 2002;70:313–317
PII: S0002-9343(06)00697-8
doi:10.1016/j.amjmed.2006.05.058
© 2007 Elsevier Inc. All rights reserved.
