A case of intravascular large B-cell lymphoma mimicking erythema nodosum: the imporightance of multiple skin biopsies

Case Details

Disease Location

Right thigh, right lower leg, the left thigh, and left lower intravascular

Personal Characteristics

62 -year-old female september 1993

Clinical Characteristics

Patient noticed lesions on legs successively for two months, the lesions were accompanied with hard, tender, intradermal or subcutaneous nodules. The first skin biopsy revealed ectatic vessels in the dermis and subcutaneous tissue with perivascular mononuclear cell infiltration. The infiltrating cells were typical physical exam showed irregularly shaped, well-demarcated, up to palm-sized, light or dark red macules with intradermal or subcutaneous, tender and hard nodules on the medial sides of both thighs and lower legs. Clinical appearance mimicked erythema nodosum, lymphadenopathy and spleno-hepatomegaly were not evident. A second biopsy from the right lower leg nodule showed a winding and ectatic blood vessel in the deep dermis with intravascular dense proliferation of the uniformly large mononuclear cells with vesicular round nuclei and high nucleocytoplasmic ratios, few large nucleoli and atypical mitosis were seen, partial thrombus was seen, there were similar cells in the slightly ectatic blood vessels in the subcutaneous tissue immunohistochemical on formalin-fixed and paraffin-embedded tissue revealed: intravascular cells were positive for l-26 and kappa light chain, and negative for lambda light chain, factor viii-related antigen and cd 24 and others (article doesn't mention 'others'). Endothelial cells were positive for factor viii-related antigen and CD24 and negative for others. Diagnosis suggested intravascular large b-cell lymphoma, clonal rearrangement of immunoglobulin heavy chain and t-cell receptor genes were not detected by southern blot analysis lab exam revealed an elevated ldh of 1200 iu/l, elevated alpha-hbdh of 732 iu/l and slightly elevated crp. Anti-htlv-1 antibody of serum was negative. Bone marrow aspiration revealed hypocellular marrow and atypical mononuclear cells that were 20-30 micrometers in diameter with vesicular round nuclei and basophilic cytoplasm with pas-positive granules, these cells accounted for 10% of the nuclear cells in the marrow, thus it was concluded bone marrow was involved there was a pigmented scar and scattered hemorrhages in the left retina via eye exam; neurological exam, gi exam, brain MRI, and CT scans of the chest and abdomen found no malignancy in may 1994, she experienced trouble walking, deterioration in intellect and incontinence of urine; neurological exams found lethargy, incomplete hemiparalysis of the right side, low or no response of the superficial reflexes for the entire body, and positive reaction of bilateral babinski's and chaddock's reflexes. Skin lesions and superficial lymphadenopathy were no present a brain MRI indicated multiple lesions that were represented by low-density areas in the t1 imaging and high density areas in the t2, a brain biopsy revealed scattered degenerated areas and large cells within the blood vessels of the brain tissue similar cells were scattered in extravascular areas of the tissue, immunohistochemical findings showed proliferating cells were positive for l-26; systemic chemotherapy (thp-cop regimen) produced no improvement of neural symptoms and patient died of respiratory failure on august 12th, no post-mortem exam was performed

Remission Characteristics

The size of the nodes decreed by about 50% spontaneously in one months and further prior to treatment the nodules completely disappeared and bone marrow exam revealed normcellular marrow and a decrease in atypical cells down to 1.5%, the serum level of ldh decreased to 600 iu/l

Treatment & Mechanisms

Proposed Remission Mechanisms

Regression may be observed in patients with other lymphoproliferative processes no major proposed mechanism…

Clinical Treatment

She received 6 courses of cyclophophamide, doxorubicin, vincristine, and prednisolone (chop regimen) from november 1993 thp-cop regimen for neural symptoms

Non-Clinical Treatment

None reported