Case report – patient 1:
A 58-year-old male patient (ex-smoker, abstinent since 30 years) was
admitted in May 2020 to our institution due to very severe
thrombocytopenia with platelet counts of 1 x 109/L
(range, 140-360 x 109/L). The patient reported
progressive petechia at the lower legs and large hematomas after minor
trauma. A few days prior to admission a dental operation was performed
with increased bleeding. Thus, complete blood count was taken. With the
exception of extremely low platelets, blood count was unremarkable. No
B-symptoms were present.
In suspicion of an idiopathic thromobocytopenic purpura (ITP) therapy
with corticosteroids (dexamethasone 40 mg/kg/body weight for four days)
and immunoglobulins 30g per day for five days was initiated. Since
severe thrombocytopenia was persistent despite combined therapy the
thrombopoetin-receptor agonist eltrombopag 50mg/day for 7 days was
added. However, there was still no response to therapy.
Flow cytometry analysis of the peripheral blood showed no evidence of a
T-cell lymphoma, but a shifted kappa/lambda ratio (0.2) with no reliable
differentiation of a monoclonal subpopulation. Bone marrow evaluation
displayed a hyperplastic, slightly dysplastic medullary pattern after
administration of corticosteroids, but no clear lymphocytic
infiltration. Flow cytometry of the bone marrow detected a small
monoclonal B-cell population (0.8%), most compatible with marginal zone
lymphoma, which could not be confirmed by immunohistochemistry staining.
Cytogenetics was normal in 20 metaphases. In fluorescence in-situ
hybridization analysis no MALT1-rearrangement was present. In addition,
a computer tomography-scan (CT) was performed, which showed large,
centrally located focal infiltrates in both sides of the lung (Figure 1,
Panels A and C) as well as a splenomegaly of 16 cm. Histopathology
evaluation after bronchoscopic biopsy revealed a BALT-Lymphoma (Figure
2). Further staging with esophageal-gastro-duodenoscopy detected a
gastric infiltration of a MALT-lymphoma without Helicobacter pylori
infection resulting in an Ann Arbor stadium IIIA.
A combined immunochemotherapy with rituximab
(375mg/m2, day 0) and bendamustine
(90mg/m2, day 1-2) was initiated. Already in response
to rituximab the platelet count increased to 121 x
109/L. Thus, the patient was discharged and the
therapy was continued on an outpatient basis for a total of six cycles,
repeated every four weeks. Contrast-enhanced CT stagings were performed
after three and six cycles showing no evidence of the disease after six
cycles (Figure 1, Panels B and D). Platelets were within normal range
during treatment without additional supportive medication or
transfusions. In addition, a gastroscopy was performed without any
further detection of suspicious results. Thus, complete remission (CR)
of the BALT lymphoma was achieved.