Lyme Misdiagnosed As Leukemia

Int J Hematol. 2007 May;85(4):323-5.

Central nervous system involvement of previously undiagnosed chronic lymphocytic leukemia in a patient with neuroborreliosis.

Kalac M, Suvic-Krizanic V, Ostojic S, Kardum-Skelin I, Barsic B, Jaksica B.


Department of Medicine, Merkur University Hospital, Zagreb Medical School, Zagreb, Croatia.


Leukemic involvement of the central nervous system (CNS) in previously undiagnosed chronic lymphocytic leukemia (CLL) is very rare. We report the case of a 62-year-old man with neuroborreliosis in which cytologic, immunocytochemical, and flow cytometry analyses revealed the presence of clonal B-lymphocytes in the cerebrospinal fluid (CSF).

After the patient received antimicrobial therapy, his meningeal symptoms cleared up, and the number of cells in the CSF decreased. Monoclonal lymphocytes were still detectable at the same percentage, however, despite systemic chlorambucil therapy. The application of intrathecal dexamethasone therapy led to the disappearance of B-cell CLL (B-CLL) cells in the CSF.

We presumed that the neuroborreliosis enabled the transmigration of leukocytes, including B-CLL cells, across the blood-brain barrier via activation of matrix metalloproteinase 9, an enzyme known to open the blood-brain barrier.

Onkologie. 2007 Nov;30(11):564-6. Epub 2007 Oct 16.

Lyme disease in a patient with chronic lymphocytic leukemia mimics leukemic meningeosis.

Schweighofer CD, Fätkenheuer G, Staib P, Hallek M, Reiser M.

Involvement of the central nervous system (CNS) is a rare complication of chronic lymphocytic leukemia (CLL) and seems to be more frequent in patients with Richter's syndrome or prolymphocytic transformation. Cases with leptomeningeal involvement reported in the literature mostly do not discuss the definition of CLL-associated meningeosis and the exclusion of neuroborreliosis.


We present the case of a 75-year-old male patient who was admitted to a rural hospital with ataxia, disorientation, and signs of progressive CLL disease. He was diagnosed of suspicious meningeosis leukemica, and treatment was started with dexamethasone for leukemic CNS involvement.


When referred to our center, careful immunophenotyping of the CNS lymphocytes as well as assessment for infectious causes of lymphocytic meningitis led to the diagnosis of Lyme disease/neuroborreliosis. An antibiotic regimen with ceftriaxone for 3 weeks resulted in complete remission of all symptoms. There was no need for CLL treatment.


In conclusion, this case report should alert clinicians that lymphocytic meningeal involvement in CLL patients accounts for the rare leukemic meningeosis only if cerebrospinal fluid cells show a predominating immunophenotype of typical BCLL cells, i.e. by flow cytometry, and if any infectious cause including Lyme disease has been ruled out.

Cutis. 1989 Apr;43(4):333-7.

Erythema chronicum migrans as the presenting manifestation of juvenile chronic myelocytic leukemia.

Inoue S, Gordon R, Berner G.

We describe the case of a 3-year-old boy who presented with typical clinical features of Lyme disease, including erythema chronicum migrans and arthritis. Over subsequent months, however, the clinical picture evolved into juvenile chronic myelocytic leukemia. To our knowledge, the combination of Lyme disease and juvenile chronic myelocytic leukemia has not been described. Dermatologic and other relevant clinical findings are presented, and the cause and effect relationship between these two rare entities is discussed.

Infection. 2007 Apr;35(2):110-3.

Seronegative Lyme neuroborreliosis in a patient on treatment for chronic lymphatic leukemia.

Harrer T, Geissdörfer W, Schoerner C, Lang E, Helm G.

We report on a patient who developed seronegative Lyme neuroborreliosis complicating chemotherapy for chronic lymphatic leukemia. After the fifth cycle of chemotherapy (FCR: fludarabine, cyclophosphamide, rituximab and prednisone) the 63-year-old patient developed night sweat, arthralgia in elbows, wrists, proximal interphalangeal joints (PIPs) and strong neuropathic pain in both legs, followed by paresthesia and hypesthesia in the feet, arms and face. Laboratory analysis revealed an elevated C-reactive protein (CRP), a slight elevation of liver enzymes and decreased IgG levels. Cerebrospinal fluid (CSF) analysis showed a lymphomononuclear pleocytosis and an elevation of protein. A broad diagnostic work-up was negative including a negative Borrelia IgG and IgM ELISA. The patient did not remember recent tick bites, but after specific questioning he recollected a transient erythema on his leg developing just before the start of the last cycle of chemotherapy. As the combination of neuropathic pain and arthralgia, the transient erythema and the lymphomononuclear pleocytosis raised the suspicion of Lyme neuroborreliosis, the patient was treated for 3 weeks with ceftriaxone. On therapy all symptoms resolved and CRP normalized. Retrospective PCR analysis of a CSF sample confirmed the clinical diagnosis by detecting Borrelia garinii DNA. This case demonstrates that in immunosuppressed patients borrelial serology may be negative and that additional diagnostic approaches (including tests for direct Borrelia detection) may be needed to demonstrate borrelial infection.

J Cutan Pathol. 2002 Mar;29(3):142-7.

Specific cutaneous infiltrates of B-cell chronic lymphocytic leukemia (B-CLL) at sites typical for Borrelia burgdorferi infection.

Cerroni L, Höfler G, Bäck B, Wolf P, Maier G, Kerl H.

Cutaneous manifestations of B-cell chronic lymphocytic leukemia (B-CLL) comprise a wide spectrum of clinicopathologic presentations. In some cases, onset of skin lesions is triggered by antigenic stimulation, and specific skin infiltrates at sites of previous herpes simplex or herpes zoster infection have been well documented. Specific skin manifestations of B-CLL can also be observed at sites typical for lymphadenosis benigna cutis (nipple, scrotum, earlobe), a Borrelia burgdorferi-associated cutaneous B-cell pseudolymphoma.


We studied specific skin manifestations of B-CLL arising at sites typical for B. burgdorferi-induced lymphadenosis benigna cutis, analyzing tissues for presence of B. burgdorferi DNA using the polymerase chain reaction (PCR) technique. Six patients with B-CLL (M : F = 4 : 2; mean age: 67.8) presented with specific skin lesions located on the nipple (four cases) and scrotum (two cases).


Clinically there were solitary erythematous plaques or nodules. Histology revealed in all cases a dense, monomorphous infiltrate of small lymphocytes showing an aberrant CD20+/CD43+ phenotype. In all cases monoclonality was demonstrated by PCR analysis of the JH gene rearrangement. PCR analysis showed in four of the six cases the presence of DNA sequences specific for B.burgdorferi.


Our study demonstrates that infection with B. burgdorferi can trigger the development of specific cutaneous infiltrates in patients with B-CLL.

Last Updated- April 2019

Lucy Barnes