All catastrophes are not catastrophic antiphospholipid syndrome

A 47-year-old Caucasian female presented with fevers and a generalized rash. She felt well until a week prior to the presentation when she started noticing a rash on her back which later became generalized. A day prior to presentation, she developed fevers (102 F) associated with nausea, vomiting, chills, and rigors as well as low back pain. A review of other organ systems was negative. She had a history of Hashimoto's thyroiditis, alopecia areata, shingles, and a positive tuberculin test 2 decades ago treated with isoniazid. She was not taking any medications or herbal supplements except levothyroxine. She drank a glass of wine a week and did not smoke or use recreational drugs. Her family history was significant for thyroid disease in her mother and sister, rheumatoid arthritis in her brother, systemic lupus erythematosus (SLE) in her maternal cousin, and pulmonary embolism (PE) in her mother. She had gone hiking three weeks before the presentation but had not noticed any insect or tick bites. She could not recollect any sick contacts though she regularly interacted with children while working as an elementary school social worker. Her blood pressure was 117/67 mm Hg, pulse 129/min, temperature 103.6 °F, respiratory rate 26/min, and saturation 92% on ambient air. On examination, she appeared ill and uncomfortable with a diffuse vesiculopapular rash involving the chest, abdomen, and extremities with sparing of palms, soles, and the face (Figure 1). She had shallow ulcerations of the buccal mucosa, injection of bilateral conjunctiva, and shotty lymphadenopathy involving the cervical, axillary, and inguinal lymph nodes bilaterally. Labs were significant for mild thrombocytopenia (platelet count of 133 10/μL) with lymphocytopenia (Table 1). A Tzanck smear did not show multinucleated giant cells. She was started on acyclovir and doxycycline. The next day, she developed worsening thrombocytopenia (platelet count 83 10/μL) prompting an evaluation by the hematology service. Prior to being evaluated, she became hypoxic requiring a high flow nasal cannula. Labs showed a platelet count of 35 10/μL with normal hemoglobin and WBC counts (Table 2). Serum LDH (810 U/L, normal 120–246 U/dL), D-dimer (>7650 ng/mL, normal <500 mg/dL) and fibrin split products (>20 μg/mL, normal <5 μg/mL) were elevated with normal fibrinogen levels (210 mg/dL, normal 173–454 mg/dL). There are different ways to approach thrombocytopenia which can help narrow down the differential diagnoses. One approach is to consider thrombocytopenia mechanistically as being due to reduced production, increased consumption (hemostatic or anatomic), or increased destruction of platelets. We also find it helpful to classify thrombocytopenia based on three clinical-laboratory aspects, by classifying it as acute versus chronic, isolated versus involving other cell lines, and as mild, moderate, or severe in severity (Table 3). Our patient had acute, isolated, moderate thrombocytopenia in the setting of sepsis, likely from a disseminated viral infection, making sepsis-related thrombocytopenia versus immune thrombocytopenia (ITP) the leading differentials. Despite an elevated LDH, her normal hemoglobin, bilirubin, and creatinine as well as an absence of schistocytes on the peripheral blood smear, made a Received: 25 January 2022 Revised: 24 February 2022 Accepted: 1 March 2022