A complete blood count revealed a hemoglobin level of 8.3 g/dl, white blood cell count (WBC) of 8,400/mm3, and platelet count of 62,000/mm3. Biochemical analysis showed elevated lactate dehydrogenase (LDH) levels of 690 IU/L. The erythrocyte sedimentation rate (ESR) also significantly increased to 48 mm/hr. Coombs tests both direct and indirect were negative along with normal bilirubin levels and thyroid function. Peripheral blood smear showed normocytic, normochromic red blood cells (RBC). We then started a bicytopenia workup after obtaining these results.

Serum protein electrophoresis was normal. Human immunodeficiency virus (HIV), Hepatitis B, and Hepatitis C tests were negative. Ultrasound and Pap smear were unremarkable. Antinuclear antibody (ANA) showed a homogenous pattern with a high titer (1:145); rheumatoid factor (RF) was positive (30 IU/ml).

A bone marrow biopsy was performed, yielding normal results and thereby excluding blood malignancies.

Further comprehensive investigations were conducted to rule out autoimmune conditions like SLE and RA, which revealed Sjögren’s syndrome antigen A (SSA-A/Ro52) positivity, narrowing our diagnostic possibilities. SSA/RO is a marker for systemic lupus erythematosus (SLE) and Sjogren’s syndrome (SS). However, in our patient’s case, the absence of a photosensitive rash and serositis ruled out SLE.

Consequently, we diagnosed the patient with SS based on the 2016 ACR-EULAR Classification Criteria for primary Sjogren’s syndrome.¹¹ Our patient meets the score of more than or equal to 4 required to diagnose Sjogren’s syndrome.

An oral dose of prednisolone (1mg/kg) was started along with supportive therapy (oral pilocarpine, artificial tear, and saliva). After a month of follow-up, her symptoms subsided along with an increase in hematological markers which is tabulated below in Table No-2.