Age: 88.0
Sex: M
Vaccine: COVID19
Symptoms: Hepatosplenomegaly, Hypotension, Hypoxia, Immune thrombocytopenia, Immunoglobulin therapy
Clinical narrative: Note 1 Nov 2021:  88 y.o. male with history of  CLL on acalabrutinib, CAD s/p stent directly admitted from Clinic on 10/26 for neutropenic fever with pancytopenia. IVIG was given on 10/27 for suspected ITP due to covid vaccine,, and patient had an infusion reaction. Increased oxygen requirements, fever, and encephalopathy requiring transfer to ICU on 10/29. Hematology is consulted for continuity of care.    # CLL # Pancytopenia # Acute DIC # Concern for HLH - Diagnosed 8/2019, peripheral blood flow revealed immunophenotype c/w CLL and FISH showed del13 and del6 - 7/2021: started acalabrutinib due to progressive symptoms and cytopenias              - Treatment on hold since 10/20 for cytopenias - Hgb 11.4 with MCV 90 on admission (baseline Hgb 12-14) -- > 8.7 - Platelets 8 on admission, s/p IVIG 1gm/kg 10/27 & 28 for suspected ITP -- > 9K with last transfusion 10/31 - Haptoglobin <30, fibrinogen 52-- > 48 with last 5 pack of cryo 10/31, INR 1.6, PTT 34, D-dimer 2211, LDH 1632-- >2536, TB 1.4, Ferritin >7500, TG 236 - WBC 2 with ANC 1100 and ALC 980 on admission -- > 23 with ANC 760  - s/p BMBx 10/28:Residual chronic lymphocytic leukemia/small lymphocytic lymphoma, interstitial pattern, involving approximately 20-30% of cellular elements  in hypercellular bone marrow (60-70%) with decreased granulopoiesis, increased erythropoiesis, dyserythropoiesis, and 3% blasts.  - 10/29: RRT called for hypoxia, agitation; transferred to ICU - started on dexamethasone for HLH from 10/29/21 - remains on high flow oxygen, and pressors   # Acute Hypoxemic Respiratory Failure # IVIG Reaction # Neutropenic Fever - Patient with low-grade fevers and dry cough for several weeks, Tmax 101-102F usually at night - CXR (10/26): bilateral small pleural effusion and bilateral opacities at the bases - IVIG reaction (10/27): RRT called for tachycardia, high-grade fever, AMS, hypotension (SBP 90s); given steroids and Benadryl. No hives, angioedema, or rash. Oxygen requirement up to 6L, patient with intermittent AMS afterwards - CT chest (10/28): bibasilar atelectasis and mild pleural effusions - CT A/P (10/28): lymphadenopathy (increased in abdomen, decreased in pelvis) and new mild-moderate hepatosplenomegaly - ID consulted, concern for disseminated fungal infection given new hepatosplenomegaly - Currently on meropenem   # Severe Dysphagia # Weight Loss # Melena - Patient reports decreased appetite noticed as early as September 2021, increasing difficulty with swallowing of solids, unintentional 10-15lb weight loss since July, and black stools - Last endoscopy/HRM in 2016 unremarkable - GI consulted, plan for EGD once patient is more stable   # AKI - Scr 1.14 on admission (baseline 0.7-0.8) -- > 4.07   # Transaminitis - AST/ALT normal on admission -- > AST 238, ALT 66   Recommendations: ?Discussed in details with the patient and the family about his previous and recent labs, imaging findings, his diagnosis, possible management options and prognosis. We explained that his HLH is not responding as we expected with the steroids, he has been on the steroids for 3 days by now. Briefly explained about use of etoposide in HLH but also expressed the concern about tolerability of chemotherapy in a frail elderly patient, in ICU, on pressors with severe cytopenias. Patient and his family understand the severity of the disease and agree with the plan. We will plan to continue the steroids for now. - Continue dexamethasone 10mg/m2 with plans for tapering as follows for suspected HLH:              - Week 1 and 2: 10mg/m2              - Week 3 and 4: 5mg/m2              - Week 5 and 6: 2.5mg/m2              - Week 7: 1.25mg/m2              - Week 8: taper to zero -   unfortunately he ultimately died of complications stemming from tx of ITP 2/2 covid vaccines given 1/28/2021, 2/22/2021, and 8/28/2021